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ANKYLOSINGSPONDYLITIS:

thefacts

Muhammad Asim Khan

OXFORDUNIVERSITY PRESS

OXFORD MEDICAL PUBLICATIONS

ANKYLOSINGSPONDYLITIS

thefacts

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thefacts

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ANKYLOSINGSPONDYLITIS

thefacts

Muhammad Asim Khan MD FACP FRCP

Professor of MedicineCase Western Reserve University

School of Medicine, Cleveland, Ohio, USA

1

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Dedication

I dedicate this book to my family (my parents, Umarand Hameeda, my wife, Mastoora, and my sons Aliand Raza), and above all to all the people like mewho suffer from ankylosing spondylitis, and to theirfamilies, as well as to their healthcare providers.

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Preface

This book is primarily intended for people with anky-losing spondylitis (AS), their family members andfriends. I hope it will also prove useful to healthcareprofessionals and organizations working with ASpatients.

As an academic doctor, a rheumatologist, myresearch interest has focused on AS and related dis-eases called spondyloarthropathies, which are alsocovered in this book. I have a more personal interestthan most researchers, because I have suffered from avery severe form of AS since I was 12 years old. Someof the problems I have faced because of this diseaseare highlighted in two recent articles (Khan, 2000,2001).

Early diagnosis and proper medical management ofAS and related diseases can help alleviate symptoms,prevent wrong treatment, enhance the future qualityof life, and help reduce the risk of long-term disabil-ity and deformity.

People with AS need to receive appropriate coun-seling, and also information about self-help issues,any potential lifestyle modification, and health edu-cation for enhancement of self-management. Thishelps them to achieve sustained health benefitswhile reducing healthcare costs and facilitatingcompliance with the recommended drug therapy andexercise regimen.

Patients who are knowledgeable about their dis-ease have more self-responsibility, comply better

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with the recommended treatment regimen, and aremore likely to make positive behavioral changes thatwill help them achieve an improved health statusand outcome in the long run. This book is intendedto add to their knowledge, and I hope that it willserve its intended purpose.

I am grateful to many AS self-help groups andorganizations for their helpful suggestions, and inparticular to Ernst Feldtkeller.

Muhammad Asim Khan MD FACP FRCP

Professor of Medicine, Case Western ReserveUniversity School of Medicine, MetroHealthMedical Center, Cleveland, Ohio 44109, USA

Preface

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Acknowledgements

I am grateful to Deutsche Vereinigung MorbusBechterew, the German AS society, and to theNational Ankylosing Spondylitis Society, the BritishAS societies, for permission to reproduce somefigures from their publications.

● Figure 5 is reprinted with kind permission fromAtlas of rheumatology, edited by Gene Hunder,Current Science, Philadelphia, 1998.

● Figure 7 is reprinted with kind permission fromStraight talk on spondylitis, published by theSpondylitis Association of America.

● Figures 8–13 are reprinted with kind permissionfrom A positive response to ankylosing Spondylitis—aguidebook for patients, produced by the RoyalNational Hospital for Rheumatic Diseases, Bath,1998.

● Figure 14 is reprinted with kind permission fromMorbus Bechterew—ein Leitfaden für Patienten, byErnst Feldtkeller, Deutsche Vereinigung MorbusBechterew (DVMB), Schweinfurt, 1985.

● Figures 15 and 20 are reprinted with kind permis-sion from Morbus Bechterew—ein Leitfaden fürPatienten, by Ernst Feldtkeller, Novartis PharmaVerlag, Nürnberg, 1997.

● Figure 16 is from Bechterew-Brief, the newsletter ofDVMB, No. 78 (September 1999), p. 15. ©Deutsche Vereinigung Morbus Bechterew,Schweinfurt.

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● Figure 17 is from Bechterew-Brief, the newsletter of DVMB,No. 56 (March 1994), p. 13–16 and from MorbusBechterew—ein Leitfaden für Patienten, by Ernst Feldtkeller,Novartis Pharma Verlag, Nürnberg 1997. © Detlef Becker-Capeller (Cuxhaven), schematic drawing by ErnstFeldtkeller (München), adapted from a similar drawing byAndrzej Seyfried in Pathologische Grundlagen derBewegungstherapie chronisch entzündlicher Gelenk- undWirbelsäulenerkrankungen, EULAR-Verlag Basel.

● Figure 19 is from Primer on the rheumatic diseases, edited byJ. H. Klippel, Edition 11, page 191, Arthritis Foundation,Atlanta, Georgia, 1997.

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1 Facts and myths about ankylosingspondylitis 1

2 What is ankylosing spondylitis? 5

3 Early symptoms 13

4 The course of the disease 19

5 Exercise and physical therapy 23

6 Drug therapy 37

7 Nontraditional (complementary, oralternative) therapy 51

8 Surgical treatment 61

9 Some later manifestations 65

10 A typical case history 71

11 Living with ankylosing spondylitis:some hints 75

12 The management of AS: an overview 87

13 The rheumatologist’s role 91

14 Radiology and diagnosis 95

15 The disease process 101

16 HLA-B27 and the cause of ankylosing spondylitis 111

thefactsCONTENTS

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17 Spondyloarthropathies 125

Appendix 1 Ankylosing spondylitisorganizations 143

Appendix 2 Glossary 151References and furtherreading 173

Index 183

Contents

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thefacts 1

Facts

• Ankylosing spondylitis (or AS for short) is achronic, progressive, painful inflammatoryrheumatic disease, which affects the spinal joints,in particular those at the base of the spine (thesacroiliac joints and the lumbar spine).

• AS typically affects young people, beginningbetween the ages of 15 and 30. The average ageof onset of the disease is 24 years, but it mayrange from 8 to 45 years.

• AS usually starts with chronic low back pain andstiffness which is gradual and insidious in onset.It can take a long time, on average about 6 years,before the correct diagnosis is made.

• Over many years, AS can result in gradually pro-gressive stiffness and limitations of spinal mobil-ity and also restricted expansion of the chest.

• In some people AS can affect other joints besidesthe spine, in particular the hip and shoulder joints.Involvement of these and other limb joints is morecommon in some developing countries, especiallywhen the disease starts in childhood.

1Facts and myths aboutankylosing spondylitis

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• About one-third of AS patients have one ormore episodes of acute eye inflammation (acuteiritis).

• AS has a characteristic appearance on X-rays,especially changes that result from inflammationof the sacroiliac joints of the pelvis (sacroiliitis).Unfortunately, this X-ray evidence may takesome time to appear. An X-ray taken in the earlyyears of the disease may be negative or indefinite(equivocal), but eventually the sacroiliac jointswill show evidence of sacroiliitis.

• The disease sometimes occurs in more than onemember of a family.

• The cause of AS is not yet fully known, but thereis an important genetic element; most peoplewith AS have a gene called HLA-B27. In peoplewith AS this gene is found in over 90% of north-ern Europeans, about 80% of Mediterraneanpeople, and about 50% of African-Americans. Inpeople without AS this gene is present in only8% of whites in the US and 2–3% of African-Americans.

• Many genes are involved, not just HLA-B27.The search is now on for these additional genesand also for the trigger factor (possibly a bacterialinfection) that starts the disease processes.

• Some AS patients may also have associated pso-riasis, chronic inflammatory bowel disease (ulcer-ative colitis and Crohn’s disease) or reactivearthritis (Reiter’s syndrome).

• There is no cure for AS yet, but the symptoms ofback pain and stiffness usually respond well tonon-steroidal anti-inflammatory drugs (NSAIDs)and a regular program of physical exercise.

2 thefacts

Ankylosing spondylitis: the facts

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• Although the course of AS is quite variable, mostpeople with the disease do well and continue tolive normal and productive lives although theymay have to modify their lifestyle or their workenvironment. For example, a manual workerdoing frequent or prolonged bending and heavylifting may have to consider a change of job.

Myths

Myth AS is rare.Fact AS affects at least 1 in 200 adults (approxi-

mately 0.5%), but its prevalence seems todiffer in various parts of the world. A studyin Germany has shown that AS affects 1%of the adult population there, making it ascommon as rheumatoid arthritis. AS is farmore common than better-known diseasessuch as leukemia, muscular dystrophy, orcystic fibrosis.

Myth AS does not affect women or children.Fact Recent studies suggest that AS is 2 to 3

times as frequent in men as it is in women.The disease may also progress more slowly inwomen. It can affect children, although bethe disease may appear initially to be slightlydifferent. Rather than back pain and stiff-ness, a child may have painful heels, knees,or hips.

Myth AS is a progressive disease that alwaysresults in a fused spine.

Fact The symptoms and severity of AS vary fromone person to another. Many people do notprogress to complete bony fusion of the

thefacts 3

Facts and myths about ankylosing spondylitis

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whole spine, because the inflammation mayease off before this can happen. For peoplewith the progressive form of AS, theinflammation does tend to spread over theyears to involve the whole spine. But,although the spine becomes more stiff orrigid, the pain in the joints of the backregresses, as inflammation is replaced by ahealing process that involves new bone for-mation. This is sometimes referred to asburning out of the disease. However, someoccasional features of AS, such as eyeinflammation (acute iritis) and heel pain,may continue to occur, suggesting that thedisease may not have gone into completeremission.

Myth Nothing can be done to help the ASpatient.

Fact Early diagnosis can prevent wrong treatmentand help set up proper medical managementthat can minimize symptoms and helpreduce the risk of disability and deformity.

Myth There has been no new major breakthroughin the treatment of AS patients who havefailed to respond adequately to the conven-tional therapy.

Fact Some recent studies have shown that suchpatients seem to respond very well to anti-TNF therapy.

4 thefacts


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thefacts 5

Ankylosing spondylitis (AS) is a chronic (progres-sive) painful inflammatory rheumatic disease thatinvolves the back, i.e. the spine and sacroiliac joints(Figure 1). The disease typically begins in adoles-cence and young adulthood, and only rarely does itbegin after the age of 45 years.• The word ankylosing comes from the Greek root

ankylos, meaning bent, although it has now cometo imply something that restricts motion (stiffen-ing) and may ultimately result in fusion. Whenthe joint loses its mobility and becomes stiff it issaid to be ankylosed.

• Spondylitis means inflammation of the spinalvertebrae; the word is derived from spondylos,which is the Greek word for vertebra, and -itis,which means inflammation. The name thereforesuggests that AS is an inflammatory disease ofthe spine that can lead to stiffening of the back.It is sometimes called just spondylitis for short,but this word should not be confused withspondylosis, which relates to wear and tear inthe spinal column as we get older.

2What is ankylosingspondylitis?

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6 thefacts


Figure 1 Sites that may be involved in AS. The mostcommonly involved sites are the sacroiliac joints and thespine. They are marked by rectangles. Other, relatively lesscommonly involved sites are hip and shoulder joints, andless often the knee joints. These sites are marked by circles.

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AS in history and literature

AS has affected people since ancient times. Onesuch sufferer was the famous Egyptian PharaohRamses II. The first definite description of AS canbe credited to an Irish physician, Bernard Conner(1666–1698). When he was studying medicine inFrance, some farmers brought him a skeleton theyhad found in a cemetery. He wrote that the boneswere ‘so straightly and intimately joined, their liga-ments perfectly bony, and their articulations soeffaced, that they really made but one uniform con-tinuous bone’ (Figure 2).

The first clinical descriptions of the disease datefrom the late nineteenth century, and the medicalinterest in AS was stimulated by a series of publica-tions in the 1890s by Vladimir von Bechterew(1857–1927) in St Petersburg, Russia. Other clinicalreports on AS were published by Adolf Strümpell(1853–1926) and Pierre Marie (1853–1940).Valentini published the earliest X-ray examinationof a patient with AS in 1899, and in 1934 Krebsdescribed the characteristic obliteration of thesacroiliac joints.

Although AS is a readily observed disorder inpeople with advanced disease, it has rarely appearedin literature. Eudora Welty mentioned it in a shortstory ‘The Petrified Man’, published in the SouthernReview of 1938–1939.

Terminology

Over the years AS has been known by many dif-ferent names, including:• spondylitis ankylosans

thefacts 7

What is ankylosing spondylitis?

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• spondylarthritis ankylopoetica• morbus Bechterew (Bechterew’s disease)• morbus Strümpell–Marie–Bechterew• Marie–Strümpell’s spondylitis• poker back.

During the first half of the twentieth century ASwas wrongly called ‘rheumatoid spondylitis’, partic-ularly in the USA, because of the mistaken beliefthat it was just a variant of rheumatoid arthritis.

8 thefacts


Figure 2 First representation of a skeleton with AS in itsfinal state by Bernard Conner, London, 1695.

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Structure of the spine

The spine consists of 24 vertebrae that are stackedone above the other and held together by strongligaments and by more than 100 joints (Figure 3). Itis divided into three main sections:• the upper part, in the neck (cervical spine) has

7 vertebrae• the middle part (thoracic spine) has 12 vertebrae• the lower part (lumbar spine) has 5 vertebrae.

thefacts 9


AtlasAxis

Cervicalvertebrae

Thoracicvertebrae

Lumbarvertebrae

Sacrum

Coccyx

AtlasAxis

Cervicalvertebrae

Thoracicvertebrae

Lumbarvertebrae

Sacrum

Coccyx

Figure 3 The vertebral column.

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Each of these sections has its own gentle curvature,and the neck is the most mobile part of the spine.The 12 ribs on either side that make up the chestwall are attached to the thoracic vertebrae in theback by joints called costovertebral and costo-transverse joints, and are attached to the breastbone(sternum) in the front chest wall by costochondraljunctions.

What is the sacroiliac joint?

The lowest (i.e. fifth) vertebra in the lumbar spinesits on a bone that forms the back of the pelvis.This bone is called the sacrum, and it looks like akeystone in the circular pelvis. It is attached oneither side to the pelvic bone called the ilium byjoints called sacroiliac joints, and by strong liga-ments (Figure 4). The front part of the pelvic bone(not shown in Figure 4) is called the pubis, and thepubic bones of the two sides form a junction in themiddle called the pubic junction (or pubic sym-physis). The lower part of the pelvic bone that bearsour weight when we are sitting down is called thegluteal tuberosity; there is one on either side, cush-ioned by the buttock.

Family history

AS does tend to run in families, and studies indi-cate that there is a genetic predisposition to it. Thiswas clearly established in 1973, when researchersfound a remarkable association of AS with a geneticmarker called HLA-B27, which is discussed in moredetail later in the book (Chapter 16). HLA-B27 is

10 thefacts


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found in 8% of the general white population of theUSA, but in more than 90% of people with AS.The prevalence of this gene is very different inother racial groups, as also discussed in Chapter 16.

thefacts 11


(a)

(b)

Figure 4 The sacroiliac joint: (a) location of the rightsacroiliac joint marked by the line separating sacrum fromilium; (b) horizontal cross-section across both right and leftsacroiliac joints—the lower part is facing the front.

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Inheriting the HLA-B27 gene does not in itselfmean that you will get AS; it simply increases theprobability. Current research is focusing onidentification of the additional genes that pre-dispose people to AS, and the activating agent orinfection that triggers the disease.

Developments in treatment

The first major advance in drug therapy in AS camewith the availability of the first non-aspirin non-steroidal anti-inflammatory drugs (NSAIDs),especially phenylbutazone, in the mid-twentiethcentury. Many other NSAIDs have since beendiscovered that are safer than phenylbutazone, butnone of them is more effective in relieving the painand inflammation of AS. The latest potential break-through is the remarkable efficacy of anti-TNFtherapy in AS patients who do not respond ade-quately to NSAIDs and other conventional medica-tion (see Chapter 6).

12 thefacts


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thefacts 13

The hallmark symptom of AS is sacroiliitis, theinflammation of the sacroiliac joints. The paincaused by sacroiliitis is usually a dull ache that isdiffuse, rather than localized, and is felt deep in thebuttock area. At first it may be intermittent or onone side only, or alternate between sides; however,within a few months it generally becomes persistent(chronic) and is felt on both sides (bilateral).Gradually the lower back becomes stiff and painful,as the inflammation extends to the spine in thatarea (lumbar spine). Over many months or years theback pain can gradually extend further up the spineto the area between the shoulder blades or even tothe neck. These initial symptoms usually start inlate adolescence or early adulthood.

Most people with AS first seek medical helpwhen the back pain and stiffness become persistentand troublesome. Their characteristic symptoms arechronic low back pain and stiffness that have comeon gradually, for no apparent reason.

The course of the disease is very variable. Somepeople with AS have only transient episodes of

3Early symptoms

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back pain with periods in between (remissions)when there are hardly any problems; others havemore chronic back pain that leads to varyingdegrees of spinal stiffness and gradually decreasingspinal mobility. However, the spine will not alwaysfuse completely: in some people the disease maystay limited to the sacroiliac joints and the lumbarspine.

The disease may sometimes be associated withinflammation of hip or shoulder joints (called thegirdle joints), or the more peripheral limb joints,such as knees, ankles, or elbows. In fact, for somepeople, the first symptoms may not be back pain butpainful girdle or limb joints. AS can be difficult todistinguish from some other rheumatic diseaseswhen there is no back pain present. However, thetypical back symptoms patients generally developlater.

Your first visit to the doctor may concern inflam-mation at some other sites, which then turns out tobe associated with AS. For example, you may haveone or more episodes of acute inflammation of theeye (acute iritis) or of the bowel (inflammatorybowel diseases such as Crohn’s disease and ulcera-tive colitis). Many people with AS can have bowelinflammation, without being aware of any intestinalsymptoms. These aspects are discussed in moredetail in Chapter 15.

Pointers to early diagnosis

Back pain in the general population is verycommon, probably only second to the commoncold as a cause of discomfort and incapacity

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prompting a visit to the doctor. It is the most fre-quent reason for temporary disability for personsunder 45 years of age, and up to 80% of Americanswill have a lower back problem of some type atleast once by age 50.

Most people with this so-called ‘nonspecific’ backpain recover within 6 months, regardless of anymedical care or intervention. It is only in a small pro-portion of people with such back pain that AS andrelated spondyloarthropathies are the underlyingcause.

Most cases of AS can be diagnosed, or at leastinitially suspected, on the basis of a good medicalhistory and a thorough clinical examination. Never-theless, there are sometimes delays and failures indiagnosis. Your doctor can help to prevent delay indiagnosis, by distinguishing back pain due to AS fromother common causes of back pain.

The back pain of early AS is usually a dull achethat is difficult to localize, felt deep in the buttock orlower back. The back pain and stiffness may beassociated with muscle spasms and tenderness in theback. The symptoms are typically worse on waking upin the morning (‘morning stiffness’) because a longperiod of inactivity usually makes the pain and stiff-ness worse. It may even be bad enough to wake youup at night sometimes. You find it necessary to exer-cise or move about for a few minutes before goingback to bed, and may have considerable difficulty ingetting out of bed in the morning. Physical activity ora hot shower helps minimize the back pain and stiff-ness, and exposure to cold or dampness may make thesymptoms worse. Occasionally, too, people maycomplain that they get fatigued easily.

thefacts 15

Early symptoms

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For some people the back symptoms may be absentor very mild, and some may complain only of backstiffness, fleeting muscle aches, or tender areas alongthe back and pelvis. The problem may occasionallybe misdiagnosed as ‘fibrositis’ or ‘fibromyalgia’.

In a thorough physical examination, the doctorshould look for the presence of sacroiliitis (by notingany tenderness elicited by direct firm pressure, orpain caused by putting physical stress on the sacroil-iac joints), and measure spinal mobility in all direc-tions, including the mobility of your neck (Figure 5).

The doctor should also check for any restriction ofchest expansion and examine your limbs for anysigns of joint inflammation and restricted range ofmotion, especially of the hip and shoulder jointswhich are affected in one-third of patients. Thelimbs and the trunk, including the whole spine, thebreastbone and adjacent ribs, and the heels, shouldbe checked for any tenderness.

The ability to bend the spine backwards and side-ways (without bending the knees), or to rotate thespine, is generally the first to be impaired. Manypeople with early AS can bend forward quite well,and even touch the ground with their fingertips,because they have good mobility in their hip joints.However, a careful examination of lumbar spinalmotion using the Schober test (Figure 5g) will oftendetect a decrease in the forward bending flexibilityof this part of their spine.

The diagnosis of AS also involves X-rays andtests to exclude other possible causes of symptoms.These are described in more detail in Chapter 14.

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thefacts 17

Early symptoms

(b)

(h) (i)

(c)

(d)

(e)

10 c

m

(a)

(f)

(g)

10 cm 15 cm

Figure 5

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thefacts 19

AS does not follow the same course in everyone:even among affected members of one family theoutcome is not exactly the same. In very early stagesthe symptoms may come and go, but in most peoplethey ultimately become more persistent. However,the lower back pain and stiffness does settle down inthe end, but by that time the upper part of yourback and the neck may have become painful andstiff as well. It is therefore very important to main-tain a good posture and prevent a stooped (bent)spine. Modern treatment can help, provided thediagnosis is made early and you comply with therecommended treatment. Most of the loss of func-tion occurs during the first 10 years, and is cor-related with the occurrence of peripheral arthritis(including hip and shoulder joints) and develop-ment of bamboo spine. The disease process of AS isdiscussed in detail in Chapter 15.

Although most of the symptoms of AS begin inthe lumbar and sacroiliac areas, they may sometimesmostly involve the neck and upper back, or present

4The course of the disease

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as arthritis in the shoulders, hips, and feet. Avariety of other problems may precede back painand stiffness in some patients, e.g. eye inflammation(acute iritis) (see Chapter 15). Eye specialists (oph-thalmologists) should always look for the possibilityof underlying AS and related diseases in someonewith this kind of inflammation. Restricted spinalmobility and decreased chest expansion without anobvious cause such as emphysema or scoliosis shouldalso alert the doctor to the possibility of AS.

AS in men and women

Until a few years ago, AS was thought to be muchmore common in men than in women. We nowknow that women frequently develop the diseasetoo, but some of them have a very mild form of thedisease which may not be as easily detected as it isin men. AS has been under-diagnosed in women inthe past. For example, in Germany only 10% of theAS patients diagnosed around 1960 were women,but this percentage has progressively increased sincethen to reach 46% among those diagnosed since1990.

There is also a significantly longer delay indisease diagnosis for female patients, but fortunatelythis delay is decreasing. For example, in Germanyin the 1950s there was, on average, a 15 years delayin diagnosis for women, but by 1975–79 it was downto 71⁄2 years.

The average age at onset of AS does not differsignificantly for men and women, but the spinefusion (ankylosis) may progress more slowly inwomen than in men. In some women, neck and

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peripheral joint involvement may be the mainmanifestation, and some may have symptoms thatresemble fibrositis (fibromyalgia) or early rheuma-toid arthritis. Functional outcome, as analyzed bystudying activities of daily living, is similar in menand women. However, when it comes to pain andthe need for drug therapy, women with AS tend tobe worse off than men. The slower and relativelyincomplete progression of spinal fusion in womenmay mean that it takes longer for pain to decreaseas a result of complete spinal ankylosis.

AS in older people

It is very rare for AS to begin after the age of 45.However, there are many people with AS whosedisease is diagnosed in old age, perhaps because theyhave had minimal symptoms over the years. Some-times their back pain may be due to osteoporosis orrelated fractures rather than to inflammation.Osteoporosis and AS in older people are discussedin detail in Chapter 9.

AS in childhood

For AS starting in childhood, i.e. up to 16 years ofage (juvenile AS), knee problems may occasionallybe the initial reason for consulting the doctor.Sometimes arthritis involving the hip, ankle, orfoot may be the first symptom. Some children mayhave mild constitutional symptoms such as malaise,loss of appetite, or mild fever in early stage of thedisease. These symptoms may be relatively morecommon in developing countries.

thefacts 21

The course of the disease

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Sites along the back, pelvic bones, sacroiliacjoints, and the chest may be tender due to thepresence of enthesitis (see Chapter 15). Some chil-dren feel pain or tenderness at the bony prominencein front of the knee, located an inch or so below theknee cap (the tibial tubercle), or heel swelling andtenderness (due to Achilles tendonitis and plantarfasciitis) (see Figure 6).

Spondyloarthropathies

AS belongs to a family of diseases that may affectthe spine and other joints, and also share manyoverlapping clinical features. This group of diseasesare called spondyloarthropathies, and they are dis-cussed in more detail in Chapter 17.

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Regular exercises are of fundamental importance inthe successful long-term management of AS. Theyhelp maintain or improve posture, chest expansion,and spinal mobility, they improve health status, andthey prevent or minimize deformity. Recreationalexercise improves pain and stiffness, and exercisingyour back eases pain and improves function. Doingsome recreational exercise at least 30 minutes perday and back exercises at least 5 days per week willimprove your health status. Sports and recreationalactivities are discussed in more detail in Chapter 12.

Formal physiotherapy is helpful, especially as asource of information about proper posture, appro-priate exercises and recreational sports, and theneed for maintaining a regular exercise program. Atleast a couple of sessions at a physical therapy unitto learn these things from a physical therapist arerecommended. A yearly follow-up by a physiothera-pist can check that you are still performing theseexercises appropriately, and also keep records of anyimprovement or worsening in physical posture, andrange of motion of your joints and spine.

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An exercise program of stretching and strength-ening is needed to keep the muscles strong and thespine mobile and erect, and to retain good range ofmovement of certain joints, particularly hip andshoulder joints. Gentle stretching exercises easestiffness and help prevent postural changes, andmuscle-strengthening exercises help in retainingproper posture. Passive stretching of the hip jointsincreases their range of movement and thusimproves function and posture.

Most people with AS feel too stiff to exercise inthe morning, although taking a warm bath beforeexercising tends to ease this discomfort. Choose atime of the day that works best for you.

The use of large Swiss therapeutic exercise ballsand group exercise sessions that include hydro-therapy are enjoyable and very helpful. In someEuropean countries, professionally supervisedspecial physiotherapy and hydrotherapy group ses-sions for AS patients have been organized by ASpatient organizations. Randomized controlled trialshave shown that physiotherapy with disease educa-tion is effective in the treatment of people with AS,and group physical therapy is cost-effective com-pared to individualized therapy.

Therapeutic exercises must be tailored to yourdegree of spinal mobility or involvement, youshould do them routinely once or twice daily. Eventhough you may not be able to do them all daily,you should do at least some of them each day. Mostpeople who comply with a comprehensive manage-ment program that depends upon a lifetime of dailyexercises can maintain satisfactory spinal mobility,and can continue to lead full and productive lives.

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Even with optimal treatment, some people willdevelop a stiff spine, but they will remain functionalif the spine fuses in an upright position.

Swimming

Swimming is an ideal exercise for those who enjoyit, because it gently uses all the muscles and is veryrelaxing. It provides aerobic exercise to enhancegeneral fitness and enhance lung capacity. A warmor even hot pool is generally most comfortable. Aheated swimming pool or spa helps to decrease painand stiffness, and therefore allows you to performexercises when it might otherwise be impossiblebecause of the pain. Low-impact exercises in thewater (swimming and water aerobics) and station-ary bicycling can help improve exercise capability,muscle strength, and range of motion.

Regular free-style swimming is considered to beone of the best exercises for people with AS, but ifyour neck is rigid it may be difficult to swim free-style. Using a snorkel may be helpful, provided youswim only under observation and near the edge of aswimming pool if it is deep. This precaution is neces-sary because someone with limited breathing capac-ity may not be able to blow the water out effectivelyif it inadvertently enters the snorkel tube.

You should be very careful not to slip on wet sur-faces in the pool area, and it is also wise to avoiddiving.

Application of heat

A warm shower or application of local heat maypromote relaxation and help in passive stretching of

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Exercise and physical therapy

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tight muscles. You should not apply local heat to anarea for more than 15 minutes at a time. Avoidareas overlying artificial joints. Keep the tempera-ture setting of the heating pad at low or mediumlevel, never on high setting. Do not lie on a heatingpad to apply heat to your back, otherwise you willincrease the risk of burn due to decreased bloodcirculation in the area that results from pressure ofyour body weight.

Spinal extension and deep breathingexercises

You can perform spinal extension exercises by lyingface down on your front and then stretching yourarms out at shoulder level and raising your chest,shoulders, arms, and head off the bed as far aspossible (Figure 6). Hold your body in that positionfor about 5 seconds and then relax, and repeat theexercise about 20 times.

The chest expansion exercise is performed bylying on your back, clasping your hands behind your

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Figure 6

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head, and extending your elbows outwards towardsthe bed while taking a deep breath. Hold the breathfor a count of 10 before exhaling and relaxing forabout 10 seconds. Repeat the exercise about 20times. Give up smoking, in order to prevent itsadverse effects on the lungs and heart.

You can combine the spinal extension and chestexpansion by performing corner push-ups, in whichyou face a corner and place your hands on theopposing walls at shoulder height. Then bend yourelbows to lean forward towards the corner with yourhead, neck, and spine fully extended, knees fullystretched and heels touching the ground (Figure 7).Take in a deep breath during this maneuver. After acount of 10, exhale while returning to the uprightposition. Repeat the exercise about 20 times, up to3 times daily if possible.

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Figure 7

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Muscle-strengthening andstretching exercises

Exercises to strengthen the extensor muscles of theback and hip can be performed in water or on land.You should try to achieve a functional range ofmotion of the hip and shoulder joint. Severe loss ofmotion of hip joints can be more disabling than thefused spine. Specific exercises such as daily stretch-ing of involved joints may be needed to improvemobility of the back, hips, shoulders, or otherinvolved joints (Figures 6–13). Physical exercisesare needed to keep your joints from getting stiff, toregain muscle strength, and prevent muscle wastingand weakness.

Figure 8b

Figure 8a

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Figure 9a

Figure 9b

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Figure 10b

Figure 10a

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Figure 10d

Figure 10c

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Figure 11a

Figure 10e

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Figure 12a

Figure 11b

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Figure 12c

Figure 12b

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Figure 13a

Figure 12d

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Figure 13b

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AS is a chronic disease, and there is currently nopreventive measure or cure for it. There is nospecial diet, and there is no convincing scientificevidence that any specific food has anything to dowith triggering the onset of AS or increasing itsseverity. A balanced diet rich in fresh fruits, andwith adequate nutrients, such as calcium and vita-mins, and a healthy lifestyle, without tobacco,alcohol or chemical addictions, are very important.

The severity of disease symptoms and the degreeof joint involvement vary greatly from one personto another. Early accurate diagnosis and appropriatetherapy may minimize years of pain and disabilitybecause with successful management it is oftenpossible to minimize spinal deformity and slowdown the progressive loss of mobility of spine andother affected joints. However, not everyonereceives early diagnosis and appropriate medicalmanagement, and some people do not continue therecommended appropriate treatment. In such cases, posture and mobility are more likely to bepermanently impaired.

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The objectives of treatment—reducing pain andstiffness, maintaining erect posture, and preservingmobility—can only be achieved if the patient takesan active part. Continuing care and proper medicalsupervision and follow-up are critical. People withAS need a kind, caring and considerate doctor witha good bedside manner, who gives patients ampletime, provides care and emotional support, andempathizes with their suffering. Because AS is achronic (long-lasting) illness, it is to your advantageto have good relationships with your healthcareproviders.

How effective is drug treatment?

Several drugs are used in treating AS. They do notcure the disease, but most minimize pain and helpmaintain mobility and function. The informationprovided below is only a guideline. You should askyour doctor and pharmacist about how and when totake any prescribed drugs and about their potentialuntoward effects.

NSAIDs

Non-steroidal anti-inflammatory drugs (NSAIDs),other than aspirin, are most often used in dosessufficient to reduce pain and suppress inflammation.The medicine must be taken as prescribed: you musttake the full anti-inflammatory dose of NSAIDsduring the active phase of the disease. Your health-care provider should emphasize this, because other-wise people may not realize how important it is anduse the drugs only occasionally, for their pain-relieving (analgesic) effect.

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More than 25 different NSAIDs are now avail-able (Table 1). They are not all equally effective,and not all of them may be officially approved bydrug-regulating agencies for use in AS in variouscountries. Responses to them differ, as do theiruntoward effects. The drug that best controls theinflammation and pain may not be the first one that

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Drug therapy

Table 1 Some well-known NSAIDs*

Generic name Brand name

Celecoxib CelebrexCholine magnesium trisalicylate TrilisateDiclofenac Voltaren, CataflamDiclofenac sodium plus misoprostol ArthrotecDiflunisal DolobidDisalcid SalsalateEtodolac LodineFlurbiprofen AnsaidIbuprofen MotrinIndomethacin Indocid, IndocinKetoprofen Orudis, OruvailKetorolac tromethamnine ToradolMeloxicam MobicNabumetone RelafenNaproxen Naprosyn, NaprelanNaproxen sodium AnaproxOxaprozin DayproPiroxicam FeldeneRofecoxib VioxxSulindac ClinorilTolmetin TolectinValdecoxib Bextra

* The brand names given here are the ones used in the US and may varyin different parts of the world. Other NSAIDs that are used relativelyinfrequently in the US include fenoprofen (Nalfon), mefenamic acid(Ponstel), and meclofenamate (Meclomen). The NSAIDs not available inthe US include nimesulide, tenoxicam, tiaprofenic acid, andphenylbutazone.

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your doctor tries; a trial period may be needed tofind the most effective NSAID for you.

It is important to emphasize that in mostinstances the NSAID does not totally relieve painand stiffness; an 80% pain relief, for example, maybe a good enough result. You may need to take theNSAID for a few days before you can tell whetheror not it is helping. Phenylbutazone (Butazolidin),one of the first NSAIDs, offers good relief of symp-toms, but it is not generally used now because thereis a potential risk of bone marrow toxicity.

Some NSAIDs need to be taken several times aday, but many longer-acting ones can be taken onceor twice daily, which makes it easier for people totake the correct dose. In the last few years, threeNSAIDs—ibuprofen (Motrin, Advil, Rufen,Excedrin, Nuprin), naproxen (Aleve, Anaprox),and ketoprofen (Actron, Orudis)—have becomeavailable over the counter in the US, so one canbuy them without a doctor’s prescription. Althoughthese NSAIDs may relieve minor aches and pains,people with AS and related diseases need to takehigher doses under a doctor’s supervision.

With appreciable relief of back pain and stiffnessat night, you should be able to get more restfulsleep. Some people may benefit from the additionof a low dose (up to 30 mg nightly) of amitriptyline(Elavil), but it may cause some untoward effects,such as dry mouth and daytime drowsiness.

NSAIDs are relatively safe drugs, but the mostcommon side-effects are stomach irritation, heart-burn (caused by stomach acid flowing back into theesophagus), indigestion, and ulcers in the stomachor duodenum. There is an increased risk of gastro-

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intestinal bleeding from ulcers, especially amongpeople over the age of 60. Other risk factors includeprevious peptic ulcer disease. You should only takeone NSAID at a time, in an adequate dose; usingmore than one NSAID at the same time increasesthe risk of side-effects without providing any addi-tive benefit.

Many of the NSAIDs need to be taken with meals,not on an empty stomach, to avoid heartburn.Additional measures needed to control heartburninclude:

• avoid foods and beverages, including alcohol thataffect the sphincter between the esophagus andthe stomach, or irritate the esophagus lining

• avoid lying down within 2 hours after eating• raise the head of your bed about 6 inches (15 cm)• stop smoking, if you are a smoker• lose weight, if you are overweight.

If you have any acute abdominal pain, severecramps or burning, vomiting, diarrhea, or blacktarry stools, seek medical attention promptly.

Medicines called H2-blockers are more effectivethan antacids to treat acid indigestion, heartburn,and ulcer pain. These drugs include cimetidine(Tagamet), ranitidine (Zantac), famotidine (Pepcid),and nizatidine (Axid). Another group of drugs calledproton pump inhibitors are even more effective;these drugs include esomeprazole (Nexium),omeprazole (Prilosec), and lansoprazole (Prevacid)

Some of the NSAIDs may impair the function ofblood cells called platelets, thereby increasing yoursusceptibility to bruising or bleeding from cuts.They can also sometimes cause fluid retention and

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Drug therapy

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mild increase in blood pressure, or some blunting ofthe effect of drugs used to treat high blood pressure.On rare occasions there may be adverse effects onkidney or liver function, and a decrease in white orred blood cell count or other signs of bone marrowsuppression. Some NSAIDs, indomethacin in par-ticular, can cause headache, drowsiness, and someimpairment of cognitive functions (a ‘spaced-out’feeling), especially in elderly people. NSAIDsshould not usually be taken during pregnancy orwhile breast-feeding.

COX-2 specific NSAIDs

Cyclo-oxygenase (COX) is a naturally occurringenzyme that exists in two forms, COX1 and COX2.COX1 can be considered the good enzyme becauseit helps in keeping intact the lining of the stomachand duodenum, in maintaining normal flow ofblood through the kidneys, and in normal plateletstickiness and aggregation. If not enough COX1 isproduced, the intestinal lining becomes vulnerableto ulceration and bleeding may occur. There mayalso be impairment of kidney and platelet function.COX2, the other variant of the enzyme, plays a rolein pain and inflammation, and its production isstimulated by inflammatory disease, infection, orinjury.

The traditional non-selective NSAIDs work byblocking the production of COX1 as well as COX2,which is why their side-effects include heartburnand stomach ulcers. However, three COX2-specific(or selective) NSAIDs are now available: celecoxib(Celebrex), valdecoxib (Bextra), and rofecoxib(Vioxx).

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They offer a new strategy for the management ofpain and inflammation since they are much safer onthe stomach and duodenum, as far as ulcer risk isconcerned, and can be taken with or without food.Moreover, they do not impair platelet function.Celecoxib has been studied in people with AS andis found to be as effective as ketoprofen, the com-pared NSAID.

However, the COX2-specific NSAIDs are nomore effective than the conventional NSAIDs, andlike them, may cause fluid retention, some increasein blood pressure, or potential impairment of kidneyfunction. Women who are pregnant or are breast-feeding should not take them.

Sulfasalazine

Sulfasalazine (Azulfidine, Salazopyrin) may be effec-tive in AS patients who have peripheral arthritisunresponsive to NSAIDs. It is one of the so-calleddisease-modifying anti-rheumatic drugs (DMARDs),also referred to simply as disease-modifying drugs orslow-acting anti-rheumatic drugs. These drugs mayslow down or perhaps stop the progress of inflamma-tory arthritis in some people, but it may take a fewmonths (which is why they are called slow-actingdrugs).

Sulfasalazine is taken with food or a glass of milk,and is available as enteric-coated tablets to decreasethe chance of stomach upset. The dose should startwith one tablet daily in the evening for the firstweek, twice daily for the second week, three tabletsdaily (one in the morning and two in the evening)for the third week, and then two tablets twice daily.Only after taking the full dose of 4–6 tablets per day

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for 4–6 months will you know whether it is going tobe of any help.

Sulfasalazine may be useful in controlling peri-pheral arthritis of AS, but has no appreciableinfluence on purely axial (spinal) disease or onperipheral enthesitis. Because it is frequently effec-tive against inflammatory bowel disease and psoria-sis, it may be especially useful for AS associatedwith those diseases. However, approximately 20% ofpatients stop the treatment because of side-effects,which include nausea, stomach upset, abdominalbloating, headaches, skin rashes, and mouth ulcers.On rare occasions sulfasalazine may cause liverproblems and abnormal white blood-cell counts dueto bone marrow suppression, and that’s why yourblood count and liver function must be regularlymonitored if you are taking this drug.

Methotrexate

People with AS with severe peripheral jointinvolvement which does not respond to NSAIDs orsulfasalazine have sometimes responded to weeklyoral methotrexate (Rheumatrex) therapy. Metho-trexate and other immunosuppressants are used inthe treatment of chronic inflammatory arthritis,such as rheumatoid arthritis and psoriatic arthritisresistant to conventional therapy. It is also a rela-tively slow-acting anti-rheumatic drug, and anyonetaking it should not expect a quick response. Likesulfasalazine, methotrexate is not a pain reliever,but it will help to relieve pain if it can first heal orcontrol the underlying inflammation that con-tributes to the pain.

It is usually well tolerated but can cause loss ofappetite, nausea, diarrhea, hair loss, cough, and

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bruising. You should tell your doctor right away ifyou get a dry cough, fever, or difficulty in breathing.Liver and blood tests and a chest X-ray are advisedbefore starting the drug, and the treatment is moni-tored for side-effects by liver tests and blood counts.Methotrexate is not suitable for people with liverand lung disease, alcoholism, an abnormal bloodcount, or active infection.

Methotrexate may temporarily reduce fertility inmen and women, but the risk appears to be verylow, as far as we can tell at present. In men there isa theoretical risk of sperm damage. Therefore, it issensible to wait for 6 months after discontinuing thedrug before attempting to start a baby. This allowsfor drug washout and avoids any theoretical risk offetal exposure.

Methotrexate may cause birth defects if takenduring pregnancy. The most vulnerable period isbetween 6 and 8 weeks of pregnancy at a criticalmethotrexate dose of more than 10 mg weekly.Breastfeeding should also be avoided while awoman is taking methotrexate.

Corticosteroids

Oral corticosteroids are powerful anti-inflammatorydrugs but cause a number of side-effects, includingosteoporosis (discussed in Chapter 9), weight gain,thinning of the skin, cataract in the eye, elevationof blood pressure, raised blood sugar, poor woundhealing, and increased susceptibility to infections.They have no therapeutic value in the long-termmanagement of the musculoskeletal aspects of ASbecause of their serious side-effects, and they do notstop the progression of the disease. Local corti-costeroid injection, however, is quite helpful in

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controlling persistent joint inflammation andenthesitis. The benefit of injection into the sacroil-iac joints is currently being evaluated.

New drug treatments

TNF-based therapy

Results of clinical trials now provide encouragingevidence of a prompt and dramatic improvement insymptoms for patients with a variety of ailmentswhen treated with drugs that block the action of anatural substance in the body called tumor necrosisfactor alpha (TNF, for short). The diseases that canbe treated include severe forms of rheumatoidarthritis, juvenile idiopathic arthritis (also calledjuvenile rheumatoid arthritis), and many otherinflammatory diseases, including Crohn’s disease,that are resistant to conventional therapy.

Anti-TNF therapy has now been found to be veryeffective in severe AS, psoriatic arthritis, and otherspondyloarthropathies that are unresponsive to con-ventional therapy. However, it can have seriousside-effects, and whether it is safe as a long-termtherapy also remains to be seen.

What is TNF?

TNF is a cytokine produced by certain inflamma-tory cells. Cytokines are messenger proteins thatplay a key role in the body’s immune response bycontrolling the production of other substancesinvolved in inflammation. The effect of TNF is topromote inflammation and also to help cells to healor repair themselves. It attaches to a cell surfaceprotein called TNF receptor on the cells belonging

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to the immune system. This receptor draws TNFinto the cell to exert its effect. When cells haveenough TNF, they release some of their TNF recep-tors into the bloodstream. These released TNFreceptors mop up any excess TNF that is circulatingin the bloodstream or is present in the tissues.

The original reason for calling this substance‘tumor necrosis factor’ was that is could inducedestruction (necrosis) of cancerous tumors in labora-tory studies. When it was first discovered it wastested for its ability to induce destruction of can-cerous tumors in animals and later in cancer patients.However, doses large enough to shrink tumors causedserious toxic reactions in cancer patients.

What is anti-TNF therapy?

If too much TNF is produced, it can damagehealthy tissues and contribute to a variety of ail-ments such as toxic shock. One way the scientistscould prevent this in laboratory animals was byadministering decoy TNF receptors that can captureexcess TNF, or by treatment with anti-TNF anti-bodies. However, when such anti-TNF therapieswere tested in human patients with toxic shock, theresults were disappointing.

TNF is also involved in triggering the inflam-matory response in many chronic inflammatorydiseases such as rheumatoid arthritis and AS.Laboratory animals genetically altered to producetoo much of this substance develop arthritis, andadministering anti-TNF antibody to these animalscan prevent the development of this arthritis.

In 1992, 20 people with rheumatoid arthritiswere treated with the anti-TNF antibody called

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infliximab (Remicade), a genetically engineeredhybrid molecule made by combining human andmouse proteins. This study provided clear-cut evi-dence of the effectiveness and relative safety ofinfliximab. Additional trials have now establishedinfliximab as a new treatment for severe rheumatoidarthritis and Crohn’s disease, although the therapydoes not provide a cure. Infliximab is now knownto be very effective in treating severe AS andrelated spondyloarthropathies which do not respondto conventional therapy. The drug is given by intra-venous infusion every month (or possibly everyother month), after the first two infusions which aregiven 2 weeks apart.

Another genetically engineered, human-derivedmolecule called etanercept (Enbrel) has a similaranti-TNF effect. It is composed of components ofthe normal human TNF receptor attached to anormal human blood protein called IgG1. It acts asa decoy TNF receptor that snags and neutralizesexcess TNF and keeps it from binding the TNFreceptors on cell surfaces. Etanercept is supplied as asterile white, preservative-free powder, which mustbe stored in the refrigerator. For use, it is dissolvedin sterile water and injected under the skin twiceweekly.

The possible down side

Infliximab and etanercept are called biologicresponse modifiers, or biologicals for short. Theywork quite rapidly, and are very effective in treatingmany types of arthritis resistant to conventionaltherapy. The systemic features of aching and fatiguetend to resolve very quickly, making people feel alot better. However, 20% of people with rheumatoid

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arthritis, the disease in which biologicals have beenstudied most, do not respond, suggesting that otherpromoters of inflammation may be at work in suchpatients.

Anti-TNF therapy is very costly (up to $13 000 ayear). Another major concern is that because thesedrugs are so new, long-term scrutiny for their possi-ble side-effects is needed. TNF plays a key role inthe body’s defense against infection by promotinginflammation and helping cells repair themselves.Long-term anti-TNF drug therapy might leavepeople vulnerable to potentially serious infections.In addition, as with other therapies aimed atmodifying the body’s immune response, there is atheoretical possibility that anti-TNF therapy maypromote malignant disease (cancer) in the long run.Doctors and patients must carefully weigh thepresent advantages against future, as yet unknown,side-effects.

Other potential new therapies

Experimental drug therapies under study for possiblebenefit in the treatment of refractory AS includethalidomide and pamidronate; the latter needs to begiven into the vein as an infusion.

Most doctors now believe that radiation treat-ment of the spine has no place in the modern man-agement of AS, because of potentially serious andeven fatal side-effects, including cancer and bonemarrow failure, which may occur many years afterthe course of radiation therapy. However, inGerman-speaking countries, radium treatment thatgives only mild radiation is still occasionally used ata few centers for treating severe AS if NSAIDs donot help. The treatment may take the form of radon

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gas inhalation, or a bath (radon dissolved in water)at some spa centers, or injection of radioactivehigh-purity radium chloride.

The present author has no experience in this areaand would not recommend this form of treatment,in part because of concerns about its long-termsafety, and because alternative, effective and rela-tively safer methods of treatment are available formanaging patients unresponsive to NSAIDs.

Storage of medications

Keep all medications out of reach of children, evenif the bottles have ‘child-resistant caps’, becausethese caps are not ‘child proof’. Do not store drugsin the bathroom cabinet because humidity and heatmay impair their effectiveness. Discard medicineswhen they reach their expiry date shown on thebottle. Make sure that the expiry date is shown onthe bottle when you buy any medicines.

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Complementary and alternative healthcare remedieshave lately become more popular. The Americanpopulation spends more than $1 billion a year onnontraditional treatments or folk remedies for arthri-tis. People use such treatments for many reasons,such as lack of adequate relief with many conven-tional arthritis medicines, or their untoward effects.Another reason is that many conventional medicaland surgical treatments are quite costly. Moreover,arthritis treatment attracts charlatans peddling‘miracle cures’.

Unlike conventional medicines, nutritional sup-plements and herbal preparations are not regulatedby agencies such as the US Food and DrugAdministration (FDA). People are therefore usingmany of these substances without any certaintyabout their precise strength, composition, and dose,and without scientifically valid proof of their safetyor effectiveness. Moreover, some practitionersproviding complementary medicine do not need tohave a license or other proof of their competency to

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prescribe such remedies or procedures. Some formsof complementary medicine may also be expensive,and it is not usually covered by health insurance.

The use of many of the complementary and alter-native treatments is based on mostly anecdotalevidence, mostly from individuals who report theirown successful use of the treatment. Scientificmethods should be applied to establish the validityof the anecdotal evidence.

Sometimes people benefit from nontraditionalremedies because of the placebo effect, and onother occasions they may experience coincidental‘cure’ because many rheumatic diseases can havecyclical spontaneous disease flare-ups and remis-sions. It is tempting to credit relief of symptoms tothe complementary medicine that, just by chance,was started when the disease was beginning to gointo remission, even though the medicine mayreally have had no effect on the disease.

A Canadian survey of people with osteoarthritisfound that many of them had used a variety of com-plementary therapies, but only 30% of them haddiscussed this with their doctors. These therapiesincluded chiropractic treatment, acupuncture,massage therapy, yoga, homeopathy, naturopathicremedies, and nutritional supplements and multi-vitamins. Three-quarters had used vitamins.

Anyone with limited spinal mobility due to ASshould avoid manipulation of their back or neck bychiropractors and masseurs, because it can be dan-gerous. Such treatment have sometimes inadver-tently led to spinal fractures and neurologicalcomplications.

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Diets

Elimination diets require you to stop eating certainfoods. One investigator has suggested the possiblebeneficial effect of a low-starch diet involving areduced intake of bread, potatoes, cakes, and pastain the management of AS. However, this diet hasnot been scientifically evaluated and there has beenno independent scientifically valid confirmation ofits overall benefit, so it is therefore not recom-mended.

Nutritional supplements that contain vitamins C,E and A, and omega-3 fatty acids are being studied aspossible treatments for arthritis. Glucosamine andchondroitin sulfate supplements are also under studyto establish if they have any beneficial effect inosteoarthritis of the knee. S-Adenosylmethionine(SAM-e, pronounced Sam-ee), a compound thatoccurs naturally in all human tissues, is another sup-plement that is being studied as a possible therapy forosteoarthritis. It has been used in Europe for years asa prescription medication for arthritis and depression,and it became available in the US as an over-the-counter supplement in March 1999.

Homeopathy

Homeopathy uses extremely diluted preparations ofnatural substances, such as plants and minerals, andscientists are skeptical about its effectiveness. Arecent study of the homeopathic treatment with‘Formica ruta’ concluded that it is not effective inAS.

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Traditional Chinese medicine

The ancient taditional Chinese system of medicine(TCM) includes herbal and nutritional supple-ments, meditation, acupuncture, and restorativephysical exercises and massage.

Herbs are the basis for many traditional medi-cines, such as aspirin, morphine, and digitalis; andpractitioners of some complementary therapiesbelieve that certain herbs have anti-inflammatoryeffects. Many of the herbal therapies that are nowused in complementary or alternative medicinewere used by the mainstream medical profession upuntil the early part of the twentieth century in thewestern world. Many of them are still consideredmainstream medicine in some poorer regions of theworld that lack modern healthcare and its effectivetherapies. Some herbs contain powerful andpotentially toxic substances that can interfere withother medications that you may be taking, so youshould talk to your doctor before taking any herbalpreparation.

The regular practice of meditation helps you toenter a deeply restful and relaxed state, with areduction in the body’s stress response, slowing ofbrain waves and heartbeat, and decrease in muscletension.

A doctor with AS has reported his personal expe-rience with Tai Chi (Koh, 1982), a traditionalChinese mind–body relaxation exercise system.

Acupuncture is based on the Chinese concept ofbalanced Qi (pronounced chee), or vital energy, thatflows throughout the body via 12 main and 8secondary pathways (called meridians), accessedthrough the more than 2000 acupuncture points on

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the human body. It is one of the oldest medical pro-cedures in the world, originating in China morethan 2000 years ago. It is believed to remove theimbalances of Yin (negative energy and forces in the universe and human body) and Yang (posi-tive energy). This brings the body into balance,keeps the normal flow of the vital energy Qiunblocked, and restores health to the mind andbody.

Acupuncture became widely known in the US in1971 when New York Times reporter James Restonwrote about how doctors in China eased his abdom-inal pain after surgery by puncturing the skin withhair-thin needles at particular locations. Althoughthe mechanism of action is unclear, stimulation ofacupuncture points may lead to release by the brainand spinal cord (via the endorphin system) ofopium-like molecules (neurotransmitters and neu-rohormones), that help to modulate pain; the samecan happen also after vigorous exercise.

Acupuncture could work due to its placebo effect.It has been shown that a real drug, naloxone (whichinhibits endorphin-producing cells in the brain),can reverse pain relief obtained by placebo (sham)painkiller; this indicates that in some cases placeboworks via the endorphin system. The Chinese claimthat acupuncture also leads to biochemical changesthat may stimulate healing and promote generalwell-being.

The World Health Organization (WHO), whichis the health branch of the United Nations, listsmore than 40 conditions for which acupuncture isused, including nonspecific back and neck pain, andarthritis. This is based on mostly anecdotal evi-

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dence, mostly from people who report their own suc-cessful use of the treatment. Scientific studies areunder way to establish the validity of this anecdotalevidence of the potential benefit of acupuncture insome forms of arthritis (see NIH, 1998).

Other therapies

Hypnosis can also be used to promote relaxationand help you cope better with pain. Another way ofachieving a restful and relaxed state is by guidedimagery, which involves creating a vivid andpleasant mental picture. For example, you might see yourself sitting on a beach on a warm day,looking at the waves and hearing them pounding on the shore. A related method called bio-feedbackinvolves using various machines that monitor one’sbody temperature, heart rate, breathing patterns,and other bodily functions, and provide feedbackthat helps you to learn how to produce these effectsand a feeling of relaxation voluntarily, without theneed for machine monitors.

Holistic medicine deals with an integrated com-prehensive overview of a physical, mental, emo-tional, and spiritual being; practitioners may suggesttherapies based on the whole person, including spir-itual and mental aspects, not just the specific part ofthe body being treated. They may advise changes indiet, lifestyle, and physical activity to help treatyour condition.

Transcutaneous electrical nerve stimulation(TENS) requires passing an electric current to nerve cells through electrodes placed on theskin. This technique is of no value for people withAS.

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Bee and snake venom are used by some alterna-tive practitioners who claim that bee venomrelieves the symptoms of rheumatoid arthritisbecause it contains certain enzymes, and stimulatesthe body to produce more corticosteroids. Thistreatment is potentially dangerous for about 10% ofthe population who have mild, severe, or even fatalallergic reactions to insect venom. Snake venom istoxic and there is little scientific support for its usein treating arthritis.

Ayurveda, the traditional Indian system of medi-cine, is also not effective in AS.

The wearing of copper bracelets, according tofolk lore, allows trace amounts of copper to beabsorbed through the skin and neutralize toxicmolecules called free radicals that can otherwisedamage tissues. These bracelets appear to be harm-less, but there is no scientific basis for their medicalbenefit.

The use of magnets as a possible therapy for jointaches and pains, and their possible untoward effects,is being investigated.

Aromatherapy practitioners believe that oilsderived from plant extracts and resins can help treatvarious illnesses when inhaled or massaged into theskin.

Dimethyl sulfoxide (DMSO) is an industrialsolvent similar to turpentine, and the industrial-grade DMSO sold in hardware stores may containharmful contaminants. Some people believe thatDMSO or its breakdown product methyl sulfonylmethane (MSM) can relieve pain and reduce swell-ing when rubbed on the skin, but rheumatologistsdo not recommend its use.

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In the end it is important to emphasize again thatthere is no rigorous scientific evidence to supportthe use of these complementary and alternativetherapies by people with AS.

Finding out about complementarytherapies

There are many quacks preying on the pain andsuffering of vulnerable or desperate people. Thesequacks may promote or promise questionable andsometimes outright dangerous treatments as ‘cures’,and may even harm people not only financially butalso medically, by keeping them away from effectivetherapies.

The National Center for Complementary andAlternative Medicine (NCCAM) recommends thatpeople should take the following steps before tryingany complementary therapy:

• Talk to your doctor first. Some forms of comple-mentary therapy that you plan to take may inter-fere with your current treatments or affect otherillnesses that you may have. Moreover, always letyour doctor know if you are already taking anycomplementary therapy because some therapiesmay potentially interact with the medicines thatyou may be currently taking as prescribed by yourdoctor.

• Ask the healthcare provider or the complemen-tary therapy practitioner about the expectedbeneficial results, risks, costs and length oftreatment.

• Check the credentials of the practitioner.Research the expertise of the practitioner or

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salesperson associated with a given treatment.Check with your local or state business bureau if you are going to buy a product from a business.

• Testimonials of other people with arthritis whohave tried a complementary or alternative treat-ment cannot prove how safe or effective thetreatment will be for others.

• Obtain objective scientific information about itat a library or through reliable Internet sources(see below).

Using the Internet

Doctors should encourage their patients to be wellinformed about their disease; patients will complybetter with treatment overall if they are properlyinformed and understand the rationale for theirtreatment (Brus et al., 1997). The Internet-savvypatient’s ability to obtain information need notadversely impact the patient/physician relationship.Much useful information can be obtained from theInternet. However, you should be selective and notbelieve everything that ‘washes ashore while you aresurfing the Net’ (Suarez-Almazor et al., 2001).There is a lot of misinformation out there too, andthis can be harmful.

You can find objective scientific information fromreliable sources, such as the National Institutes ofHealth (NIH) and MEDLINE Plus at www.nlm.nih.gov/medlineplus. Appendix 1 gives contact details forsome AS organizations, which are also a usefulsource of information (see also page 152).

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Joint replacement (arthroplasty)

Total hip arthroplasty (THA) gives very goodresults, and to a large extent prevents partial ortotal disability from severe hip disease. People whoare about to undergo THA should be in goodgeneral and dental health.

Infection can be a serious complication, but withadvances in the use of antibiotics and other tech-nical aspects, the occurrence of infections has beenmarkedly reduced in recent years. Bacteria cantravel through the blood and infect a total jointimplant, both in the early postoperative period and for some years following implantation; the most critical period is the first 2 years after jointreplacement. Dental and endoscopic procedures can cause temporary circulation of bacteria in theblood, so people with artificial joints under-going these procedures (e.g. cystoscopy andcolonoscopy) may need prophylactic antibiotics tominimize the risk of infection affecting the replacedjoint.

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Prophylaxis

People who are not allergic to penicillin should begiven amoxicillin 2 grams by mouth 1 hour before adental procedure (or cefazolin 1 gram or ampicillin2 grams by injection 1 hour before the procedure ifthe patient cannot take oral medications). Peoplewho are allergic to penicillin can be given clinda-mycin 600 mg by mouth 1 hour before the dentalprocedure, or by injection if they cannot take oralmedications. No second doses are recommended.

The American Heart Association’s suggestedregimen is 3 grams of amoxicillin 1 hour before and1.5 grams 6 hours after the initial dose. For someoneallergic to penicillin, then erythromycin may begiven, 1 gram 1 hour before dental treatment and500 milligrams 6 hours after the first dose.Clindamycin may be used an alternative.

Other surgical procedures

Severe spinal forward curvature (kyphosis) used tooccur in some people with severe AS, but its occur-rence should now be very uncommon if the diseaseis diagnosed at an early stage and treated appropri-ately. Severe kyphosis can be surgically corrected forsomeone so bent that they cannot look straightahead, or are hardly able to eat. However, thissurgery carries a relatively high risk of paraplegia. Ifsurgery is too risky, special prism spectacles can helppeople to look ahead while walking, but they arenot easy to use. Spinal surgery may also be neededfor stabilization of spinal fracture.

Heart complications, such as leaky heart valve orsevere slowing of the heart may require valve

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replacement or the placement of a cardiac (heart)pacemaker. Scarring (fibrosis) and cavity (cyst)formation in the upper part (apex) of the lung arerare complications of AS that are not easy tomanage; surgical removal of tissue may sometimesbe required.

Anesthesia in people with AS

The anesthesiologist may have difficulty in passing abreathing tube down the trachea (windpipe) so thatthe airway can be maintained during general anes-thesia for surgery. This is a potential problem inanyone with a rigid spine, especially if you also haveforward stooping of the neck and a reduced jaw-opening capacity. An instrument called a fiber opticlaryngoscope helps in putting the breathing tubedown the trachea. However, someone with extremeneck deformity may require a tracheostomy. Post-surgical lung complications are also more likely,owing to severely restricted chest wall movement.

Lumbar spinal anesthesia or other alternatives togeneral anesthesia, such as epidural anesthesia, maybe possible for some surgical procedures (e.g. totalhip joint replacement surgery). However, lumbarpuncture for spinal anesthesia is often not possiblein AS patients with a fused lumbar spine.

Do not assume that healthcare providers are fullyaware of all the limitations due to AS. You shoulddiscuss any concerns or apprehensions with thesurgeon, and arrange a preoperative consultationwith the anesthesiologist. The anesthesiologistshould examine you beforehand to find out yourlimitations, and also allay any concerns you may

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Surgical treatment

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have. This should preferably be done in your hos-pital room, before you are taken to the operatingroom, and before you are given the anesthetic pre-medications that dim your alertness of mind.

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Osteoporosis

Osteoporosis (porous bone) is a very commonaccompaniment of aging and therefore a commoncondition, among the general population, but itposes particular problems for people with AS, as wewill see later in the chapter. It is a major problemfor close to 30 million US citizens, 80% of themwomen, although it is a potentially preventableillness. One out of 2 women and 1 in 8 men overthe age of 50 will have an osteoporosis-related frac-ture in their lifetime.

Osteoporosis is characterized by low bone massthat leads to an increased susceptibility to fracturesof the spine, hip, wrist, ribs, and other bones. It isoften called the ‘silent disease’ because there maybe no symptoms until the bones become so weakthat a fall or sudden strain causes a fracture of oneor more bones of the limbs or the spine. Fractures ofthe spinal vertebrae can be in the form of compres-sion (collapse) fractures, and these may lead to lossof height, back pain, and the stooped posture called

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dowager’s hump. In a patient with osteoporosis,usually an elderly woman, the hump occurs in theupper back (thoracic kyphosis), and the spinal cur-vature may look superficially like AS.

An average woman acquires 98% of her totalskeletal bone mass by about age 20 and can lose upto 20% of her bone mass in the first 5 years aftermenopause. The best defense against developingosteoporosis in later life is to build strong bonesduring childhood and early adulthood by taking abalanced diet rich in calcium and vitamin D, fol-lowing a healthy lifestyle with no smoking, andperforming regular weight-bearing exercise.

Significant risk of osteoporosis has been reportedin people of all ethnic backgrounds, but it is morecommon among whites and Asians, and whitewomen after age 65 are twice as likely as African-American women to get fractures. Specialized bonedensity tests can detect osteoporosis before a frac-ture occurs, and can also predict your chances ofbone fracture in the future. Tests conducted atappropriate intervals can measure rate of bone lossand monitor treatment benefit.

Osteoporosis is responsible for more than1.5 million fractures annually in the US, with anestimated national direct expenditure (hospitals andnursing homes) of $14 billion annually (and rising).People over 50 years of age have an average 1 in 4chance of dying in the year following a hip fracture,and among those who survive there is 1 in 4 chancethat they will require long-term care afterward. Awoman’s risk of hip fracture is equal to her combinedrisk of breast, uterine, and ovarian cancer.

Osteoporosis is often thought of a disease of oldpeople, or women past the age of menopause.

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However, it can strike at a younger age in peoplewith predisposing (risk) factors, which include adiet low in calcium; chronic use of certain medica-tions (e.g. corticosteroids); being female, thin, orhaving a small frame; a family history of osteo-porosis; an inactive lifestyle; smoking; excessiveintake of alcohol; propensity to falls; anorexianervosa, and (for men) low testosterone levels.

Drug therapy for osteoporosis

Bisphosphonates such as alendronate (Fosamax)and risedronate (Actonel) are very helpful, and aremore widely used than treatment with calcitonin(Miacalcin). Calcium tablets may be needed if thecalcium intake in your diet needs to be supple-mented.

For women after the menopause the femalehormone estrogen helps to prevent or slow downosteoporosis. Brand names include Premarin (with-out progesterone), Prempro (with progesterone),Estratab (esterified estrogen), and others. Raloxi-fene (Evista) is the first in a new class of drugscalled selective estrogen receptor molecules(SERMs) that slow bone loss like estrogens do, butwithout some of estrogen’s untoward effects on thebreast and uterus. Therefore, raloxifen can be analernative choice for women at increased risk forcancer of the breast or uterus. However, like estro-gens, it is associated with increased risk of bloodclots and stroke.

Spinal fracture in AS

Recent studies indicate that osteoporosis can alsooccur in many people with AS in early stages of

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Some later manifestations

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their disease. It can be a result of inflammation inthe early stages of AS, as well as a result of immobil-ity in the later stages of the disease. In advanced ASthe spine usually has a low bone mass, i.e. it isosteoporotic. This structural deterioration, alongwith immobility due to bony fusion, makes thespine fragile and very susceptible to fracture.

People with AS are five times more likely to getspinal fractures than the general population. Thesefractures may follow a relatively minor trauma,especially in people with long-standing AS that hasresulted in a fused spine. They usually affect thelower neck (cervical spine). The two commonestcauses are falls and motor vehicle accidents.

The pain associated with spinal fractures may beoverlooked, or wrongly attributed to exacerbationof the underlying AS. The best early clues to spinalfracture may be an acute or unexplained episode ofback pain, even in the absence of a history of physi-cal injury, that is aggravated by movement and maysometimes be associated with localized spinal ten-derness.

There may be neurological signs and symptoms asa result of the fracture. The displaced ends of thefractured cervical spine (neck) compressing thespinal cord may cause quadriplegia (weakness orparalysis of all four limbs), the most dreaded compli-cation of AS. Isolated or multiple vertebral com-pression fractures without displacement may alsooccur.

If you have a fused spine it is wise to carry asuitable personalized information card. The cardshould state that your spine, including your neck, isfused as a result of AS, and that you are therefore

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much more prone to spinal fracture due to any fallor motor vehicle accident, even after a relativelytrivial injury. The card should include your name,address, and phone number, a photograph (includ-ing a picture showing the spinal deformity), yourblood group type, a list of medicines you are taking,any allergy history, and contact details of yourdoctor.

Inflammation of the discs in the back (spondy-lodiscitis) may sometimes occur without any physi-cal trauma or infection. This mostly occurs in themid-thoracic spine, is usually without any symp-toms, and is relatively more common in people withAS severe enough to involve the neck.

Neurological problems

As well as quadriplegia or paraplegia resulting fromspinal fracture, other neurological problems mayoccur (although they are rare). For example, theremay be a gradual loosening of a joint at the junctionof the skull and the neck as a result of inflamma-tion; this condition is called spontaneous subluxa-tion of the atlantoaxial joint.

Rarely, patients with advanced AS may haveproblems resulting from gradual scarring of the cov-ering at the lower end of the spine that entraps thelower spinal nerves. The resultant symptom com-plex is called cauda equina syndrome. (The namecauda equina means horsetail, so named because thelowermost spinal nerves slope downward as a bunchbefore they leave the vertebral column.) Symptomsmay include urinary retention and incontinence,fecal incontinence due to decrease rectal tone,

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sexual dysfunction, saddle anesthesia (so calledbecause of loss of skin sensation over the part we siton), and pain and weakness of the legs.

Other problems

Some people may get kidney dysfunction due totreatment with NSAIDs or other drugs used to treatAS, especially if they have underlying kidneydisease, perhaps due to diabetes or high bloodpressure. A complication that has now become veryrare, especially in North America, is amyloidosis ofthe kidneys, which used to be the most commoncause of kidney dysfunction in patients with AS.Rare occurrence of IgA kidney disease (glomeru-lonephritis) has been reported in some countries.

The uncommon heart and lung complicationshave been mentioned previously (see pages 62–3).

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Adam, a 26-year-old college student, was recentlyseen with back pain. He had been quite well up until18 months ago, when he started having chronic lowback pain and stiffness. He initially felt the pain inhis buttocks for a few months, and then it progressedto involve the low back area as well, and was associ-ated with back stiffness. Now his symptoms of backpain and stiffness are worsened by prolonged sitting,and at night, as well as when he wakes up in themorning. His back symptoms are worse when he firstgets out of bed but they start easing up afterabout 40 minutes, or after physical activity or exer-cise, and after a hot shower. In the last 3 monthsAdam has noticed pain in the chest (rib cage) that isaccentuated on coughing or sneezing.

He has no history of chronic diarrhea, skindisease, eye inflammation or injury to his back. Hisfather was killed in a car accident at the age of 30,and he is an only child. His paternal uncle has hada stiff back and neck for many years.

On physical examination, Adam was found tohave tenderness over sacroiliac joints, the lumbar

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spine, anterior chest wall, and right jaw joint(temporomadibular joint), as well as limitation ofmotion of his lumbar spine. His chest expansion onfull inspiration was normal, and the rest of hisphysical examination was also normal.

Because these clinical findings indicated a strongprobability of AS, an X-ray of the pelvis wasordered. The presence of bilateral sacroiliitis on theX-ray confirmed the diagnosis of AS.

He was prescribed an NSAID to be taken twice aday with food, and was encouraged to stay active,swim regularly if possible, and follow a regularexercise regimen. His illness was explained to him,and he was given counseling and provided with apamphlet that gives further information about AS.Julian is computer-literate, so he was also given theInternet addresses of reputable self-help groups andorganizations for AS patients.

When he was seen at a follow-up visit 2 weekslater, Adam’s symptoms were already much better.Assessment of pain, physical function, spinalmobility (including chest expansion), duration ofmorning stiffness, presence of any inflamed peri-pheral joints, and enthesitis are critical elementsthat will be followed over time by the medicalpersonnel caring for him. Laboratory tests, such asC-reactive protein (CRP) and erythrocyte sedi-mentation rate (ESR), and occasionally muscu-loskeletal imaging (changes on X-ray pelvis andspine) will also help his doctors to assess andmonitor the activity and severity of his disease.

Adam asked a lot of questions about AS andpossible treatments, and he had already accessedmany websites and other information sources.

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Because he takes an intelligent interest in hisdisease, he is more likely to follow the recom-mended exercise program and to comply with hismedication and follow-up schedule.

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A typical case history

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If you have physical limitations due to advancedAS, there are many devices to help performdaily tasks: walking canes, special chairs and desks,special shoes, and devices that assist in putting onsocks or stockings and shoes, or for scratching orapplying soap on the back, etc.

Avoiding falls

• Always wear a good pair of skid-resistant shoes.• Use grab bars in the shower and toilets, shower

seats, raised toilet seats, and floor lighting atnight.

• Avoid slippery surfaces and loose carpets.

Posture

• It is important to sleep on a firm bed to maintaina good resting posture at night. You shouldpreferably make a habit of sleeping on your back,to prevent the hip joints and the back frombecoming bent (Figure 14a). Avoid a pillow

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under your knees because that will increase thetendency to muscle and tendon shortening.

• Avoid a saggy mattress or a waterbed. A suitableboard (made of plywood or chipboard) can be putbetween the mattress and the bed frame to makethe bed more firm.

• Avoid using a pillow if possible, or use one justthick enough to allow a horizontal position ofthe face to prevent pain from overextension ofthe neck.

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Figure 14 Recommended sleeping positions: (a) A flatsleeping position opposes the tendency of curvature. If yourhead would fall into over-extension because your thoracicspine is already curved, a small pillow of just the right thick-ness under the back of your head may make the positioneasier. Avoid too thick a pillow. (b) Lying ‘face down’ (on yourstomach) is also a favorable position. (c) If lying on your frontis no longer possible, a stable position lying on your side (the‘recovering position’, is a good alternative.

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• You should only lie on your side for short periods,if possible.

• You should also practice lying prone (with theface down), e.g. for 5 minutes or more beforegetting out of the bed in the morning, and alsobefore going to bed at night (Figure 14b). Or youcan lie on your back across your bed with yourlegs over the side and knees bent.

• People with AS need to practice good posturehabits at all times, and should be taught aboutdynamic, resting, and occupational postures.

Dynamic posture

• Be aware of how you are standing, and try tomaintain an erect tall posture, with the spinekept as straight as possible.

• Avoid any tendency to slump forward.• Splints, braces and corsets are generally not

helpful and are not advised. Some form ofbracing may be necessary on rare occasions, e.g.after injury to the back or neck, but only on therecommendation of a doctor who is experiencedin the management of AS patients.

• Perform appropriate muscle-strengthening exer-cises regularly, as advised by your doctor.

Occupational posture

• Analyze your habitual and work postures andmodify your working positions to maintain agood posture. For example, a drafting table withtilting work surface (Figure 15) may be betterthan an ordinary office desk for writing andreading, and avoiding stress on the neck.

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• Avoid physical activity that places prolongedstrain on your back and neck muscles, and pro-longed stooping or bending.

• Alternate between sitting and standing posi-tions to perform jobs that take a long time tofinish.

• Maintain a good posture while sitting, and avoidsitting for prolong periods, especially in low softsofas and chairs.

• During your mid-day break at work, lie flat for afew minutes, and do some corner push-ups tostretch the back (Figure 7). Try to lie face downon your stomach for part of the time.

• A daily routine of deep breathing and spinemotion/stretching exercises may minimize thefusion, and at least preserve better posture. Do

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Figure 15

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deep breathing exercises at frequent intervalsduring the day.

Thus proper sitting, sleeping, walking, and workingpositions, coupled with appropriate exercises, helpmaintain good posture and chest expansion.Because hip and shoulder joints are often affected,you should exercise the range of motion of thesejoints even before you observe any symptoms orlimited motion there.

Family life

• People with AS generally have a very fulfillingand productive life. You can raise children justlike anyone else because the disease usually doesnot interfere with family life.

• Fertility, pregnancy, and childbirth are usuallynormal.

• Although pregnancy does not usually affect thesymptoms of AS, there may be restrictions oncertain kinds of drug therapy during pregnancyand breast-feeding. You should discuss the use ofany drug at these times with your doctor.

• Problems with family life may sometimes arise as aresult of severe back pain, spinal deformity orlimited spinal mobility; especially in women whohave severe involvement of the hip joints withmarked pain and limitation of joint movement.You should discuss these problems with yourdoctor. Patients with severe hip involvementbenefit from total hip joint replacement surgery(see Chapter 8).

• A very useful and informative publication titledStraight talk on spondylitis, published by the

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Spondylitis Association of America, is highly rec-ommended for additional advice. It also discusseshousework, dressing and grooming, child bearingand child care. See Appendix 1 for contact details.

Sports and recreational activities

• Sports and recreational activities that encouragegood posture as well as arching of the back(extension) and rotation of the trunk are recom-mended. These include walking, hiking, swim-ming, tennis, badminton, cross-country skiing,and archery.

• Volleyball and basketball (with specially adaptedrules) are excellent sports for people with AS as

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Figure 16 Volleyball with specially adapted rules aspractised in local groups of AS organizations (here of theGerman AS society) is an excellent sport for people with ASas it combines movement with stretching.

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they combine movement with stretching (Figure16). However, not everyone can tolerate jarringactivities.

• If you have neck involvement you need to bemore careful, and follow safety instructions.

• Sports activities that require prolonged spinalflexion, including golfing, bowling, and long-distance cycling, may be inadvisable.

• Body contact sports (such as boxing, rugby,soccer, American football, and hockey), anddownhill skiing, are also not recommendedbecause of their greater potential for injury.

• Stationary bike exercises are good, but thehandlebars must be properly adjusted so that youdo not lean forward while exercising. This exer-cise is especially good for general cardiovascularconditioning, strengthening the leg muscles, andexercising the hip and knee joints.

• Aerobic exercises with machines that enhanceback, leg, and shoulder extension are helpful, butyou should avoid undue stress on the neck.

Car driving

• You may find difficulty driving if you haveimpaired mobility of your neck. In particular, itmay be difficult to back the car into tight parkingspaces because you cannot turn and twist yourback and neck to look behind you.

• Special wide-view mirrors fitted to the car can bevery helpful. Have some practice sessions drivingand backing up the car in an open area tobecome comfortable using these mirrors. A smallhand mirror may be of use in special situations in

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avoiding ‘blind spot’.• Use seat belts and head restraints so that sudden

slowing or stoppage does not jerk the spine,including the neck. Remember that the stiff neckof an AS patient is more vulnerable to injurythan a normal neck. The top of the car seat’shead restraint should be level with the top of theyour head, and the restraint should be adjustableand as close to the back of your head as possible.

• Avoid bucket seats.• A disabled driver parking permit may be appro-

priate for anyone who can’t walk very far, but thisis usually not a problem for most people with AS.

• If you have a painful and stiff spine, and havedifficulty driving a long distance, stop after anhour or two at some appropriate place, and getout of the car to stretch your back and walkaround for a few minutes.

• The Ankylosing Spondylitis InternationalFederation (ASIF) has published a booklet fordrivers with AS. See Appendix 1 for contactdetails.

Impact of AS on employment andearning capacity

• Most people with AS are able to cope well, con-tinuing a very productive and active lifestyle.

• Your employer’s tolerance for a flexible work sched-ule or a working environment adapted to yourneeds can have a great impact on whether or notyou are able to get or keep a job. Read the chapter‘Staying employed’ in the book Straight talk onspondylitis for more information.

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• It may be helpful to alter your positions at work,e.g. use short rest periods to perform backstretching exercises during work hours, especiallyif your work involves prolonged sitting or stand-ing. This can be arranged on consultation withthe employer.

• Avoid prolonged stooping and heavy lifting; thework surface should be at a proper height toavoid bending.

• If your current job involves excessive strain onthe back because you have to work in a crampedor bent position, you may have to think of achange of job. Vocational rehabilitation agenciesare available to provide guidance.

• In a Norwegian disease outcome study of 100people with adult onset AS, just over half wereemployed in full-time work after a mean diseaseduration of 16 years. Stopping work was associ-ated with low levels of education, female sex,recurrent acute iritis, bamboo spine, and thepresence of other concommitant non-rheumaticdiseases. After more than 20 years of disease,more than 80% of the people surveyed still com-plained of daily pain and stiffness, and more than60% needed to take their anti-rheumatic medica-tions daily.

• In the past some people with AS used to get sostooped that they could not even look straightahead. Some forward stooping of the neck andcurvature in the upper back is still commonlyobserved after many years of the disease. Lookingphysically different from the rest of the popula-tion can present psychological problem, but mostpeople are able to come to terms with this.

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• Severe disease that results in complete spinalfusion in bent position and severe limitation ofchest expansion, especially when there is also con-tracture of the hip or shoulder joints, may shortenlife span because the function of the heart andlungs may be adversely affected and there is agreater risk of spinal fractures.

Health-related quality of life

• Health-related quality of life is based on yourperception of the net effects an illness has onyour life. It is commonly based on your symp-toms, physical functioning and ability to work,psychosocial functioning and interaction, un-toward effects of treatment, and direct andindirect medical and financial costs.

• Although people with AS are troubled with pain,stiffness, and limited spinal mobility, most ofthem remain in employment. A recent study at arheumatology referral center in Germany indi-cates that people with AS have a degree of pain,disability, and reduction in well-being similar topatients with rheumatoid arthritis, a more severetype of arthritis. However, such referral centersare likely to see patients with more severedisease, so their results many not apply to every-one with AS.

• In a recent survey of 175 AS patients (68%male, mean disease duration of 23.7 years, meanage 51 years) the most common quality of lifeconcerns were about stiffness (90%), pain(83%), fatigue (62%), poor sleep (54%), appear-ance (51%), side-effects of medications (41%),and concern about the future (50%). Few

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patients in this survey reported problems withsocial relations or mood.

• There are many recently published journal articleson employment, disability and quality of life ofpatients with AS. See ‘References and furtherreading section.

Depression

Depression is not uncommon in people with anychronic painful illness that impairs quality of life,and that includes AS. Depression is a treatabledisease that has many underlying causes, and someindividuals are genetically prone to it. Symptoms ofdepression include:

• loss of pleasure in activities that were onceenjoyable

• persistent feeling of sadness, emptiness, decreasedenergy, tiredness, and anxiety

• frequently feeling helpless, worthless, guilty, andhopeless, or feeling irritable and restless

• disturbed appetite (loss of appetite or tendencyto overeat)

• disturbed sleep (difficulty sleeping, waking up tooearly, oversleeping, sleeping too little or toomuch)

• difficulty in concentrating, thinking, remember-ing, or decision-making

• sometimes persistent physical problems (e.g.headache, abdominal pain) not responding totreatment

• thoughts of ending life by committing suicide.

If you have any of these symptoms you shoulddiscuss them with your doctor so that appropriatetreatment can be provided.

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Additional information on depression is availablefrom the National Institute of Mental Health(www.nimh.nih.gov) or the American PsychiatricAssociation (www.psych.org).

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• There is currently no preventive measure or acurative treatment for AS, but in most peoplethe disease can be very well managed. Early andmore precise diagnosis leads to earlier and morerational or effective therapeutic interventions.

• Splints, braces, and corsets are generally nothelpful and are not advised.

• There is no special diet, and there is no evidencethat any specific food has anything to do withtriggering or increasing the severity of AS.

• Fertility, pregnancy, and childbirth are usuallynormal in women suffering from AS.

• NSAIDs are very effective in relieving pain andstiffness in most (80%) of the patients with AS.They should be used regularly and in the fulltherapeutic anti-inflammatory doses during theactive phase of the disease. Patients should bemade aware of this, since otherwise they may usethe NSAIDs only occasionally, for their pain-relieving effect.

• The individual responses to the various NSAIDsmay vary from one person to another, as do the

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side-effects, so it is worthwhile searching out thebest NSAID for each individual.

• When the disease is not adequately controlled byNSAIDS, or for people who are intolerant ofsuch drugs, other medications may be needed,especially in those with peripheral arthritis,inflammatory bowel disease, or psoriasis.

• Newer drugs that neutralize a factor in the bodycalled TNF are very effective, but as yet theirlong-term side-effects are unknown.

• Oral corticosteroids (cortisone) have no beneficialeffect in the long-term management of AS becauseof serious side-effects, and they do not halt progres-sion of the disease. Persistent joint inflammationmay sometimes respond quite well to a local corti-costeroid injection.

• Regular exercise is of fundamental importance inpreventing or minimizing ankylosis (stiffness)and deformity. Spinal extension exercises anddeep breathing exercises should be done rou-tinely once or twice daily.

• Smoking should be avoided.• People with AS should walk erect, keeping the

spine as straight as possible, and sleep on a firmmattress using a thin pillow, just thick enough toallow a horizontal position of the face to preventpain from overextension of the neck.

• Physical activity that places prolonged strain onback muscles, such as prolonged stooping orbending, should be avoided.

• Formal physiotherapy is of value for learning theproper posture, appropriating exercises and recre-ational sports, and maintaining the exerciseprogram. Group exercise sessions that include

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warm water exercises (hydrotherapy) are veryhelpful.

• Regular free-style swimming is considered to beone of the best exercises for people with AS.

• People with limited spinal mobility due to ASshould avoid manipulation of their back or neckby chiropractors or masseurs because this can bedangerous for anyone with diminished spinalmobility. Such treatments are known to haveinadvertently led to spinal fractures.

• People with AS may have difficulty driving a carbecause of impaired neck mobility, and may findspecial wide-view mirrors helpful.

• There are many AS self-help and support groupsthat enlist enthusiastic patient co-operation,provide information about the disease and adviceabout life and health insurance, jobs, workingenvironment, wide-view mirrors and other usefulitems (see Appendix 1 for contact details).

• Total hip joint replacement (arthroplasty) givesvery good results, and prevents partial or totaldisability from severe hip disease. Vertebralwedge bone resection may be needed to correctthe severe stooping deformity that may occa-sionally occur, although this surgery carries arelatively high risk of paraplegia. Heart complica-tions may require pacemaker implantation oraortic valve replacement.

• Radiation treatment of the spine has no role inthe modern management of AS.

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Who is a rheumatologist?

Rheumatologists are physicians uniquely educatedand trained to diagnose and treat arthritis and otherdiseases of the joints, muscles and bones, such as ASand related diseases. In the US, a rheumatologist isa board-certified internist (internal medicine spe-cialist) or pediatrician who has had an additional2–3 years of specialized rheumatology training. Mostof these physicians become certified in rheumatol-ogy after passing another board certification exami-nation.

Board-certified rheumatologists are thereforehighly trained specialists in diagnosing and treatingarthritis and other rheumatic diseases. Manyrheumatologists based in academic or hospitalrheumatology units help to train other doctors andallied health professionals, as well as providingpatient care. They are also involved in conductingclinical and basic scientific research to enhance our

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understanding and management of rheumatic dis-eases. Most rheumatologists, however, are in privatepractice, and some of them have clinical affiliationswith academic medical centers.

Interdisciplinary co-operation

The well-established rheumatology units or centersinclude not only rheumatologists, but also highlytrained allied health professionals such as specialistnurses, physiotherapists, occupational therapists,and medical social workers. The rheumatologistswork closely with other health professionals such asorthopedists and physiatrists, podiatrists, psychia-trists, psychologists, and dieticians.

The rheumatologist’s most important role is todecide on the diagnosis and recommend the rightkind of management for your disease. For thosereasons a rheumatologist will ask about yourdetailed medical history and then carry out a clini-cal examination, sometimes ordering blood testsand X-rays in order to decide how best to treat you.The doctor should also explain the illness and itslong-term impact, and an appropriate treatmentplan for the future.

You should not be afraid to ask any questions andto ask for any pamphlets, leaflets or other informa-tion to help you gain better insight into yourdisease. Do not hesitate to bring someone with youto the rheumatologist’s office if you prefer.

The people with AS most likely to follow aregular exercise program are those who attend arheumatologist, believe that the exercise is ofbenefit, and are well motivated and educated.

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Consistency rather than quantity of exercise is ofutmost importance. It is the doctor’s job to relieveyour pain and stiffness, and your job to performregular exercises and to maintain a reasonably goodposture. You should see your doctor for periodicfollow-up appointments in order to maintain goodhealth.

Many patients with AS may need to be seen by arheumatologist over an extended period of time,rather than being cared for by their primary caredoctor (general practitioner).

If you are unhappy with or have doubts aboutyour treatment, it is quite appropriate to ask for asecond opinion from another consultant.

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Radiology

Conventional X-ray is generally quite helpful indiagnosing AS and distinguishing it from otherdiseases (differential diagnosis); the sacroiliac jointsin people who do not have AS will either be normalor show only some degenerative changes, but noerosions typical of sacroiliitis will be present.

The clinical diagnosis of sacroiliitis, however,may be difficult, especially in the early stages,because the sacroiliac joints are deep and virtuallymotionless, and there may be no obvious tendernesson direct pressure over the joint. A presumptivediagnosis of AS can be confirmed by finding thecharacteristic changes of AS on an X-ray, becauseinflammatory involvement and resultant damage ofthe sacroiliac joint is usually present by the timeyou seek medical attention. Finding bone erosions,narrowing or fusion of the sacroiliac joints on an X-ray confirms the presence of the disease. Radio-graphic (X-ray) evidence of sacroiliitis is requiredfor definitive diagnosis, and is the most consistent

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finding. Radiography can also detect progressivebony fusion of the spine in later stages of thedisease.

Because the onset of the disease is usually pre-ceded by a long latent period and a diagnosis isneeded to ensure proper and timely treatment, safeand relatively cheap techniques are needed todetect sacroiliitis with a high degree of sensitivityand specificity. A simple anterior–posterior X-ray(‘AP view’) of the pelvis is usually sufficient fordetection of sacroiliitis. However, such an X-ray cansometimes be normal or show only equivocal(unclear) changes in very early stages of the disease(when the structural changes in the joint are stillmostly limited to the joint lining (synovial mem-brane) and the cartilage). In this situation, a mag-netic resonance imaging (MRI) scan, possiblyenhanced by the injection of a chemical calledgadolinium, appears to be the method of choice forthe early detection of sacroiliitis.

MRI can also be used for early detection ofinflammation (enthesitis) at other sites, because itcan show the early changes in cartilage and theunderlying bone. Moreover, unlike X-rays, MRI usesno ionizing radiation and is therefore a useful tool,especially in young people, but it is very costly.

The use of MRI has led to a decreasing use ofanother radiographic imaging method called com-puted tomography (CT) to detect sacroiliitis. CTprovides a better but costlier detailing of bone andjoint changes than a conventional X-ray, and is notcommonly needed in the diagnosis of AS.Moreover, there is greater radiation exposure fromCT than conventional X-ray of the pelvis.

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Laboratory findings

Laboratory tests may not be of much help, and thereis no single blood test that can specifically diagnoseAS, i.e. there is no diagnostic or confirmatory test.However, some blood tests may contribute to thediagnosis of the disease, or correlate with its severityor clinical presentation.

A simple but non-specific blood test called anESR (erythrocyte sedimentation rate) is one of theindicators of inflammation. This test may help todetect the presence of severe inflammation, andmay be of some use in determining, for example,whether the back pain is the result of inflammationor is the more common mechanical or nonspecifictype of back pain or strain. However, less than 70%of people with AS have a raised ESR value, evenwhen there is active inflammation. Moreover, thistest is influenced by a variety of other factors, suchas anemia, age, body weight, pregnancy, and the sexof the individual tested. In a normal young man theESR is usually less than 20 mm. Another test ofinflammation is called CRP (C-reactive protein);this is less likely to be influenced by extraneousfactors.

There is no association with a blood test calledrheumatoid factor (associated with rheumatoidarthritis) or antinuclear antibodies (associated withlupus). Therefore, AS and related spondylo-arthropathies are sometimes listed under the termseronegative spondyloarthritis.

Laboratory analysis of the joint (synovial) fluidobtained by joint aspiration (arthrocentesis) orbiopsy (obtained by a needle or by arthroscopy via

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an instrument called arthroscope) does not markedlydistinguish AS from other inflammatory rheumaticdiseases.

The possible use of HLA-B27 as an aid to diag-nosis is discussed in Chapter 16.

New York criteria

The current criteria for the diagnosis of AS, knownas the modified New York criteria, are shown inTable 2.

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Table 2 The generally accepted criteria for AS (modifiedNew York criteria)

1 Low back pain of at least 3 month’s duration improved byexercise and not relieved by rest

2 Limitation of lumbar spinal motion in sagittal (sideways) andfrontal (forward and backward) planes

3 Chest expansion decreased relative to normal values for thesame sex and age

4 Bilateral sacroiliitis grade 2–4 or unilateral sacroiliitis grade 3 or 4

Definite AS if criterion 4 and any one of the other criteria is fulfilled.Note: These are classification criteria used for case definition and areprimarily designed for research purposes.

Other causes of back pain

There are many possible cause of back pain, but byfar the most common is mechanical deterioration ofthe spine. This can take many forms, but is oftenrelated to the intervertebral discs. In childhood thecentral part of these discs consists of over 85%water; there is a slow but steady decrease with aging,

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down to about 60% by the age of 80 years. As aresult the volume of the disc decreases, leading tonarrowing of the disc space, causing buckling of thesurrounding ligaments (annulus fibrosus and spinalligaments), and formation of a bony spur (osteo-phyte) at the edges of the spinal vertebral bodies.Clinical back pain related to disc degenerationincreases with age, and is accelerated by mechanicalstress.

Ankylosing hyperostosis, also called Forestier’sdisease or diffuse idiopathic skeletal hyperostosis(DISH), can cause excessive new bone formationalong the spine and some other sites. This can resultin a stiff spine that may be confused with AS. Otherdiseases that may be confused with AS includeosteitis condensans ilii, Paget’s disease (of thepelvis and spine), and Scheuermann’s disease. Thespread of cancer to the pelvis and the spine, as wellas some chronic spinal infections, can also presentas back pain.

A bone-thinning disorder called osteomalacia,which results from dietary deficiency of vitamin Dand lack of adequate skin exposure to sunlight, ormay be a result of chronic kidney failure, can causeback pain and may be mistaken for AS or relatedspondyloarthropathies. Osteoporosis can also causeback pain.

Another illness that can cause confusion is a veryrare disease of unknown cause, known as SAPHOsyndrome (because of its salient features: synovitis,acne, palmoplantar pustulosis, hyperostosis, andaseptic osteomyelitis). This disease causes bonedamage that sometimes affects the sacroiliac jointsor the spine.

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What happens in AS?

As explained in Chapter 3, the disease usuallybegins as an inflammation in the sacroiliac joints.When these joints become inflamed they causepain that you can feel not just over the joints butdiffusely over the buttock (gluteal) area. The sacro-iliac joints usually become tender on direct firmpressure in the early stages, but the pain and ten-derness gradually get less over the years as thesacroiliac joints become fused and replaced bybone. When the inflammation spreads to involvethe lumbar spine, you will be aware of low backpain and stiffness.

The inflammation and pain can result in musclespasm and tenderness, as well as stiffness of the back.There is a natural tendency to stoop forward to mini-mize the symptoms, because backward stretching isuncomfortable. This can gradually lead to irreversiblebad posture, because if the inflammation is notresolved the body begins a gradual repair process thatresults in further limitation of back motion due to

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scarring (fibrous tissue formation) and bone remodel-ling (Figure 17a, b, c).

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(a) (b) (c)

Figure 17 The effects of AS on posture: (a) A healthyperson standing erect: Note the hollow lower (lumbar) backand the inclination of the pelvis. Also shown, in a schematicdrawing (slightly exaggerated), the transmission of bodyweight vertically downward (arrow) through the hip joints(black), oblique to the plane of the pelvis. The center ofgravity is vertically in line with the hip, knee and ankle joints.(b) A person with a moderately advance stage of AS. Notethe upright position of the pelvis and elimination of thelumbar hollow (i.e. straightening of the lower back). Thewhole static equilibrium is changed; there is some forwardstooping of the neck, and the beginnings of upper thoracickyphosis. (c) A person with very advanced AS, withincreased upper thoracic kyphosis and fixed forward stoop-ing of the neck. Note the flexion contracture of the hip jointsand the flexed knees, to keep the gaze horizontal. Chestexpansion is limited, so the diaphragm must be used forbreathing, which makes the abdomen look more prominent(‘rubber ball belly’). © Detlef Becker-Capellar.

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Enthesitis

The inflammation in AS tends to start at the placeswhere joint capsules, ligaments or tendons areattached to bone, resulting in pain or tenderness atthese sites. The name enthesis is given to thesesites, and the inflammatory lesion is called enthe-sitis or sometimes enthesopathy.

The doctor should check for pain and tendernessalong the back, pelvic bones, sacroiliac joints, andthe chest, looking for the presence of enthesitis.There may be heel swelling and tenderness either atthe site of insertion of the Achilles tendon to thecalcaneus (heel bone), or at the site of the attach-ment of the plantar fascia to the same bone at thebottom of the heel (see Figure 18). The medicalnames for these conditions are Achilles tendinitis,and plantar fasciitis respectively.

A process of healing and repair, which followsthe enthesitis phase, results in gradual limitation ofback motion due to scarring and subsequent bone

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Figure 18

Site ofenthesitis

Site of enthesitis(plantar fasciitis)

Achillestendon

Tibia

Talus

Midtarsal joint

Retrocalcanealbursa

Subtalarjoint

Calcaneus

Subcutaneousbursa

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formation. This process may, after many years, leadultimately to complete spinal fusion. Any clinicalexamination to look for the presence of AS musttherefore include a thorough examination of spinalmobility in all directions (Figure 5), Chapter 3.

The inflammatory changes affect the superficiallayers of the ligament (annulus fibrosus) thatsurrounds the disc, especially at its attachment to thecorners of the vertebral bodies, resulting in increasedbone density (sclerosis) of these corners, seen on X-ray as shiny corners (Figure 19). The bone at thesecorners may subsequently disappear, and this mayultimately result in squaring of the vertebral bodies.Gradually a thin layer of vertical bony outgrowths atthe edges of the vertebrae bridges the gap betweenthe two adjacent vertebral bodies, replacing thesuperficial layer of the annulus fibrosus of the disc.This intervertebral bony bridging that surrounds thedisc is called a syndesmophyte (Figure 19).

At the same time, inflammatory changes andslowly progressive bony fusion may be going on inspinal joints called the apophyseal or facet joints(Figure 19). Thus in someone with severe diseasethe inflammatory process of the spine may gradually,after many years, result in complete fusion (also

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Figure 19

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called bony ankylosis) of the whole spine. The X-ray of the spine may ultimately look like abamboo and is sometimes called bamboo spine.Spinal osteoporosis (discussed earlier) is also fre-quently observed among such patients, partly as aresult of the lack of spinal mobility and aging.

The inflammation of the joints between the ribsand the spine (the costovertebral and costotrans-verse joints), and at the junction of the ribs to thebreastbone in front of the chest (the costochondralareas), can result in chest pain and tenderness. Thispain can be accentuated by coughing or sneezing.Over the years there may be a gradual decrease inchest expansion. Therefore, some people maypresent to the doctor with chest pain and tender-ness, or complain of inability to expand their chestfully on deep inhalation, or shortness of breath onexertion. The doctor should check not only for lim-itation of mobility (in all directions) of the spine,including the neck, but also for any restriction ofchest expansion (Figure 5f).

Involvement of non-spinal (limb)joints

The hip and shoulder joints, the so-called girdlejoints, are affected in one-third of AS patients. Thehip joint involvement usually affects both sides(bilateral) and is gradual in onset; the pain isusually felt in the groin, although you may feel it inthe knee or the front of the thigh on the same side.The hip joint involvement is more common inchildhood or adolescence (juvenile AS) when thedisease starts. Involvement of the shoulder joint isgenerally relatively mild.

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There is a gradually destruction and thinning ofthe joint cartilage that cushions the bones of thejoints, and this is accompanied by gradual limitationof joint motion. This can give rise to a characteristicrigid gait, with the patient keeping the knees a littlebent in an attempt to maintain an erect posture.

In later stages of AS some contracture of the hipjoints is not uncommon. For someone whose spine,including the neck, is rigid, involvement of the hipsjoints is more crippling and can lead to greater dis-ability, but total hip joint replacement can mini-mize those limitations.

Involvement of peripheral joints, other than hipsand shoulders, is quite infrequent except in ASpatients who have associated disease, as discussedlater. Moreover, such an involvement is rarely persis-tent or destructive, and usually tends to resolvewithout any residual joint deformity. Episodes ofinflammation of the jaw joint (temporo-mandibularjoint), occur in about 10% of patients, and causepain, tenderness, or some limitation in fully openingthe mouth.

Involvement of other structures

Spondylitis can also affect structures adjacent to thejoints, such as tendons (thick cords that attachmuscles to bone) and bursae (small sacs betweenbony prominences and the overlying moving struc-tures such as skin, muscles, or tendons). Inflamma-tion of these structures results in tendinitis andbursitis.

Some patients complain of fatigue and gettingtired easily. There may be wasting (atrophy) and

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weakness of thigh and buttock muscles due to theirlack of use, especially people with advanced hipjoint involvement. Many such patients have diffi-culty standing up from a squatting position, andmay need to hold on to something in order to getup.

Eye inflammation

One or more episodes of acute (abrupt) inflamma-tion of the eye can occur in one-third of all ASpatients at some time in the course of their disease.This is called acute iritis or anterior uveitis. Iritis isan inflammation of the iris, the colored part of theeye that surrounds the pupil, and anterior uveitismeans inflammation of the iris as well as the adja-cent inner layers of the eye (ciliary body) used forcontrolling the function of the lens (Figure 20).

Acute iritis may occur before the onset of AS or even when the disease is otherwise in ap-parent remission. It usually presents as pain,redness, difficulty looking directly at bright light,

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Figure 20

Ciliary body

Cornea

Lens

Vitreous

Choroid

Sclera

Retina

Optic nerve

Iris

White of eye

Pupil

(Light)

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and excessive tearing in the involved eye. Therecan be recurrent episodes of acute iritis, but eachsuch episode typically affects one eye only. Theremay also be some blurring or impairment of visiondue to a build-up of inflammatory cells in the front part of the eye. If it is left untreated iritis may have permanent effects on your eyesight, so it requires prompt consultation with an eyespecialist (ophthalmologist) for diagnosis and treatment.

Acute iritis can usually be easily managed withdilatation of the pupil and use of corticosteroid eyedrops for a few weeks. You may need to use darkglasses temporarily to decrease your sensitivity tobright light. Occasionally, systemic corticosteroids(orally or by injection) or other drugs such as TNF blocking drugs or immunosuppressives may beneeded for a few people with severe iritis that has not responded adequately to conventionaltreatment.

Other associated problems

People with AS should be carefully evaluated forany bowel inflammation, and heart, lung, orneurologic complications. Studies have disclosedthe presence of chronic inflammatory boweldisease, such as ulcerative colitis and Crohn’sdisease, in a large number of AS patients, some ofwhom may have minimal or no bowel symptoms.

In 2–5% of people with AS, usually after manyyears of the disease, there may be heart involvementin the form of inflammation and scarring. This canaffect the heart’s electric conduction system, andlead to slowing of the heart rate (heart block); or

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scarring and dilatation of the aorta (the majorartery), as it comes out of the heart, and of its valve,may result in a leaky aortic valve (aortic valveincompetence). The trouble may, in some patients,be serious enough to require a pacemaker or anaortic valve replacement. Impaired relaxation of theheart muscle, without affecting its ability to con-tract to pump the blood out, may also be seen insome people with AS.

People with AS who have no symptoms of lungdisease do nevertheless get functional lung impair-ment (documented by lung function testing)because of restricted chest expansion. Therefore,some of them may take longer to recover fromsevere influenza, bronchitis or pneumonia. Youshould avoid smoking, and discuss with your doctorabout the possible need for vaccines againstinfluenza or pneumonia. Scarring (fibrosis) of theupper part (apex) of the lung (apical fibrosis) is arare complication.

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We do not yet know the precise cause of AS, orwhat triggers it. Along with the other spondyl-arthropathies, it shows a strong association with agene called HLA-B27. The disease is most likelycaused by multiple predisposing factors, includinggenetic and non-genetic (environmental) factors.Infections are suspected to be possible environmen-tal triggers. AS may be triggered by gut infectionwith Klebsiella bacteria, but the evidence is circum-stantial, and more convincing proof is needed.Some of the other spondylarthropathies, particu-larly reactive arthritis (Reiter’s syndrome), can betriggered after an episode of bowel infection by bac-teria, or by infections of the genitourinary tract.

There is substantial evidence that HLA-B27 hasa direct role in enhancing genetic susceptibility toAS. However, having the HLA-B27 gene is not aprerequisite for AS, and people without HLA-B27can also get the disease. Additional genetic factorsmay influence disease susceptibility, expression orseverity; for instance, genes that are suspected tocause susceptibility to psoriasis, ulcerative colitisand Crohn’s disease, and possibly other genes yet to

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be discovered. AS patients have an increased fre-quency of mild gut inflammation, even though theyhave no intestinal symptoms or any clinicallyobvious inflammatory bowel disease (IBD). Follow-up studies of such patients indicate that a smallpercentage of them will develop clinically obviousCrohn’s disease. This suggests that these patientshad a sub-clinical form of IBD when they first pre-sented with AS. The presence of this gut inflamma-tion does not show any association with HLA-B27.These findings support the existence of a commonlink between gut inflammation and AS, indepen-dent of HLA-B27. Similar findings have also beenobserved in patients with other spondy-loarthropathies.

What is HLA-B27?

HLA stands for human leucocyte antigens. Theseare cell surface proteins that vary from person toperson. Their function is to help the body fightillness by presenting peptides (a few amino acidslinked together) derived from foreign proteins (e.g.viral or bacterial), or from the body’s own proteins,to T lymphocytes and other cells of the immunesystem. HLA are the products of genes located onchromosome number 6; the loci (where the genesare located) are given the letters A, B, C, D, and soon.

The HLA genes and their products, i.e. the HLAmolecules, are grouped into two broad classes calledHLA class I and class II. HLA-B27, or simply B27for short, is so called because its gene is located atthe B locus belonging to the HLA class I group andis assigned the number 27. Many varieties of these

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genes at these various loci exist in the general popu-lation, so it is very difficult to find two unrelatedindividuals possessing an exactly identical combina-tion of these variations. Any cell that is infected,e.g. by a virus, will usually display on its surfacepeptide antigens of viral origin, in addition to self-antigens, in combination with HLA class I mole-cules, such as HLA-B27. The presence of the viralpeptide antigens with the HLA molecule activatesCD8+ cytotoxic T cells specific for that peptideantigen to destroy the infected cell.

The role of HLA-B27 in disease predisposition

A greater prevalence of AS is observed in HLA-B27-positive first-degree relatives of AS patientsthan in HLA-B27-positive random controls. Thissuggests that AS is probably genetically heteroge-neous, i.e. there are other genetic predisposingfactors as well as HLA-B27. However, the evidencefavors the gene for HLA-B27 being the majorgenetic susceptibility factor responsible for AS. Themore disease-predisposing genes you inherit themore likely you are to suffer from AS, but most likelyit still requires some, as yet unknown, environmental(i.e. non-genetic) trigger for the disease to start.

Although people who are born with the HLA-B27 gene are more predisposed to AS or one of therelated spondyloarthropathies (i.e. they are morelikely to suffer from these diseases), most of themremain unaffected. It is important to emphasize thatthere are far more people in the general populationwith HLA-B27 who never get AS than those whodo. Even in families where one member has the

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disease and the HLA-B27 gene, most of theirbrothers and sisters will remain unaffected evenwhen they have the same gene.

Perhaps the HLA-B27-positive person destinedto develop spondyloarthropathy may be exposed tocertain gut organisms that partially imitate HLA-B27 in ways that lead the bacterial antigens tobecome immunogenic and somehow trigger thedisease. The HLA-B27 protein itself or the peptidebound to and derived from HLA-B27 may have apathogenic role.

Inheritance of HLA-B27

Each of us has 46 chromosomes in the nucleus of ourcells, and each chromosome is a tiny thread-likestructure that contains a set of genes. We derive 23 ofour chromosomes from one parent and the other 23from the other parent. Autosomes is the name givento the 22 of these pairs of chromosomes that areunrelated to the sex of the person; they are assignednumbers 1 through 22, based on their size. Theremaining two chromosomes are assigned the lettersX and Y, and they are the sex chromosomes. Eachfemale has two X chromosomes and each male hasan X and a Y chromosome. The father contributes aset of 22 autosomes and an X or a Y chromosome tothe offspring, while the mother contributes the otherset of 22 autosomes and the X chromosome.

Everyone has two HLA-B genes (one on eachchromosome 6), and someone is said to be HLA-B27 positive if B27 is the gene present at either oneor both of these HLA-B gene locations.

• If an individual has inherited B27 from bothparents so that the B27 gene is present at both of

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the HLA-B gene location (B27 homozygous)then all of that person’s children will inherit B27.

• If one parent has HLA-B27 at one of these HLA-B gene locations, as is the case for 8% of peopleof Western European extraction, then there is a 1in 2 chance that their offspring will inherit thisgene.

• The likelihood of both parents possessing theHLA-B27 gene is less than 7 per 1000. There isthen a 1 in 4 chance that the offspring from sucha marriage will inherit B27 from both parents(B27 homozygous), a 1 in 2 chance of inheritingthe B27 gene from only one parent (B27 hetero-zygous), and a 1 in 4 chance of not inheriting theB27 gene at all.

Genetic counseling

Because of this genetic predisposition, it is notunusual for more than one person in a family to beaffected with AS or related diseases, and it ishelpful for the doctor to know this family history.A person with AS (who has a >90% chance ofpossessing the HLA-B27 gene if he or she is ofWestern European extraction) may ask, ‘What is therisk of my children developing it, and can anything bedone to prevent this?’

Children who inherit HLA-B27 from the B27-positive parent with AS (and on average 50% willinherit the B27 gene) carry a risk of up to 1 in 4 ofdeveloping the disease themselves during their life-time.

Thus, most children with the B27 gene do notdevelop the disease, and the 50% of children wholack the gene carry no virtually increased risk unless

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genes for other diseases that also predispose to AS(such as psoriasis and inflammatory bowel disease)are present in the family.

If the person with AS does not possess HLA-B27(a <10% chance if he or she is of Western Europeanextraction), then the risk of disease occurrenceamong the children may not be increased at all,unless genes for other diseases that also predisposeto AS (as mentioned above) are present in thefamily.

The person with AS, who has a >90% chance ofpossessing the HLA-B27 gene, may ask, ‘Should Ihave all my children tested for the HLA-B27?’ The answer is no, because among the 50% of thechildren who are expected to be positive, an over-whelming majority (>80%) remain unaffectedduring their lifetime. Moreoever, the parents andthe healthcare providers may get ‘HLA-B27-itis’:knowing that the child has HLA-B27, the parentsand the healthcare providers can worry unneces-sarily; and symptoms unrelated to AS may bewrongly attributed to the fact that the child hasinherited the gene. Thus the child may get a wrongdiagnostic label of AS, even though he or she is anunaffected individual who happens to possess anormal gene called HLA-B27. Even a child whoremains totally healthy may suffer indirectly infuture if the information about the HLA-B27 testresult enters their medical records, and thusbecomes available to health insurance agencies, orfuture potential employers, who may misuse suchinformation.

If a child of an AS parent develops symptoms orsigns that you suspect may be due to AS or anotherHLA-B27 associated disease, you should point out

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all the child’s symptoms to their doctor, who shouldpreferably be a pediatric rheumatologist. When it isappropriate the doctor can utilize HLA-B27 typingas an aid to diagnosis.

HLA-B27 testing in diseasediagnosis

AS can almost always be readily diagnosed on thebasis of history, physical examination and X-rayfindings, and therefore HLA-B27 typing is notnecessary for disease diagnosis. A knowledge of thepresence of HLA-B27 can sometimes be valuable asan aid to diagnosis, although the prevalence of HLA-B27 (Table 3) and the strength of its association withAS vary markedly in different ethnic and racialgroups. For example, only 50% of African– Americanpatients with AS possess HLA-B27, and it is close to 80% among AS patients from Mediterraneancountries.

Thus, AS and related diseases can also occur inpeople who do not have HLA-B27. Therefore, anegative test result for B27 does not, in itself,completely exclude the presence of the disease.Moreover, a positive test result in itself does notmean that someone has the disease, because theHLA-B27 gene is present in a significant percentageof the healthy general population.

However, the test can be useful for a doctor whounderstands the principles of probability reasoningand uses it only in a toss-up clinical situation. Inother words, the doctor may think that there is a40–60% likelihood that the patient has AS, and thesacroiliac joint X-rays are either normal or showequivocal (not very definite) changes. Moreover, its

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clinical usefulness is influenced by the patient’sracial and ethnic background. Typing for HLA-B27should not be considered a routine, diagnostic,confirmatory, or screening test for AS in patientswith back pain in the general population.

Research on HLA-B27 and relatedtopics

Prevalence of HLA-B27 in world populations

HLA-B27 is not uncommon in the general popula-tion, but its prevalence varies among differentethnic/racial groups throughout the world. This isshown in Table 3. The numbers are rounded off forsimplicity, and indicate percentage prevalence inthe general population. For example, in the USAapproximately 8% of whites and 2–3% of African-Americans posses this gene, but it is much morecommon among the native Americans.

In general, AS and related diseases are morecommon in populations with a relatively higherprevalence of HLA-B27, such as among the Inuitand Eskimos, and the converse is also true (Table 4).HLA-B27 and AS are both absent from Australianaboriginal populations of unmixed genetic ancestry.However, there are some exceptions to this general-ization; e.g. AS is virtually absent in certain WestAfrican populations even though HLA-B27 ispresent in up to 6% of them.

Different types of HLA-B27

So far, 25 different types of HLA-B27 (namedB*2701 to B*2725) have been distinguished; mostof them are quite rare. The presence of the differentHLA-B27 subtypes also differs markedly among the

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Table 3 Percentage prevalence of HLA-B27 in differentpopulations throughout the world.

Native American linguistic population groupsEskimo-Aleut Eskimos and Inuit 25–40Na-Dene Tlingit, Dogrib, and Navajo 20–35Amerind Bella co-ola 26

Yakima and Pima 18–21Cree, Zuni and Chippewa 11–14Papago, Hopi and Havasupai 7–9Mexican Mestizo 3–6Central American natives 4–20South American natives 0

North and central Asiatic linguistic population groups

Chukchic Siberian Chuckchis 19–34Siberian Eskimo 40

Uralic Ural mountain natives 8–15Samis (Lapps) 24

Altaic Siberians: Yakuts 17–19Tofs 13Buryats 3–6

Japanese 1Ainu (native Japanese) 4Koreans, Uygurs and Mongolians 3–9Uzbeks, Kazakhs and Turkic

populations 3–8Sino-Tibetan Chinese (mainland) 2–6

Chinese (overseas) 4–9Tibetans 12

Caucasoid populations Ugro-Finnish 12–18

Northern Scandinavians 10–16Slavic populations 7–14Western Europeans 6–9Mediterranean Europeans 2–6Basques 9–14Gypsies (Spain) 16–18Arabs, Jews, Armenians, 1–6

and IraniansPakistanis 6–8Indians (Asian) 2–6

Other Asiatic populations

South-east Asians Vietnamese 9Khmer, Taiwanese aborigines

and Filipinos 5–8

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various world populations. For example, HLA-B*2705 is the most common subtype among whitepeople, and the Siberian and North Americannative populations, whereas HLA-B*2704 is themost common subtype among the Chinese andJapanese populations, HLA-B*2706 (B*2722) is themost common subtype among Indonesians, andHLA-B*2703 is only observed among WestAfricans or people with African ancestry.

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Table 3 (contd)

Indonesians, Malaysiansand Thais 5–12

Micronesians Nauru 2Guam 5

Melanesians Papua New Guineans 12–26Vanuatuans and

New Caledonians 18–23Ouveans 11Fijians 4–6

Polynesians Hawaians, Samoans andMarquesas Islanders 2–3

Maoris 0–3Tokelau and Society Islanders 0Rapanui (Easter Islanders) 0Australian aborigines 0

North and West African populations

North Africans Arabs 3–5Berbers 2Ethiopians 1

West Africans Gambia and Senegal 2– 6Mali 10

Equatorial and Southern African populationsPygmies 7–10San (Bushmen) 0Bantu

Nigerians 0Zimbabweans 0South African Xhosas 0–0.3Zaireans 0–0.7

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Table 4 Recent prevalence studies of AS and related spondyloarthropathies

HLA-B27 Prevalence Prevalence of spondyloarthropathies frequency of AS (%) (including AS)

in general in general in B27-positive in general in B27-positive population (%) adult population adult population adult population adult population

Eskimos (Alaska) 40 0.4 2.5Eskimos(Alaska 25–40 1.6 2–3.4 4.2and Siberia) and ChukckiSamis (Lapps) 24 1.8 6.8Northern Norway 10–16 1.4Mordovia 16 0.5Western Europe 8 0.2Germany (Berlin) 9 0.9 6.4 1.9 13.6

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Association of different types of HLA-B27 withAS

The presence of AS or related spondyloarthro-pathies has been noted in individuals born with anyof the common HLA-B27 subtypes so far known.However, two subtypes seem to be, at the most, onlyvery weakly associated with disease. These areHLA-B*2722 (formerly mistyped as HLA-B*2706),which is found in south-east Asian populations, andHLA-B*2709, a rare subtype observed in Italianpopulations, primarily on the island of Sardinia.

Other research studies

If a cell is infected, by a virus for example, it willdisplay on its surface small protein molecules calledpeptides, of viral or self-origin, in combination withHLA class I molecules, such as HLA-B27. Thepresence of the viral peptide antigens with the HLAmolecule activates CD8+ T cells (cytotoxic T cells,which are destructive to cells) which are specific forthat antigen. Certain types of HLA are moreefficient in defending against certain infections, butat the same time they may make the individualmore vulnerable to developing certain other infec-tions or diseases. For example, an individual bornwith HLA-B27 is able to mount a better responseagainst many viruses (as compared to others bornwith HLA genes other than HLA-B27), but theyare somehow more likely to suffer from AS orrelated spondyloarthropathies.

HLA class II molecules are found on cells, such as macrophages, which present antigens to theimmune system and are therefore called antigen-presenting cells. When these cells ingest bacteriaor their products, or are infected by bacteria, they

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display peptide antigens on their surface, includingthose derived from bacterial proteins and toxins, incombination with HLA class II molecules. CD4+(helper) T cells specific for these peptide antigensthen help mount an immune response against theinfection.

Laboratory rats and mice have been raised thatcarry the human HLA-B27 genes. These so-calledHLA-B27 transgenic rats and mice express HLA-B27 on their cell surface. They have been veryhelpful in finding out how HLA-B27 may predisposehumans to AS and related spondyloarthropathies.The HLA-B27 transgenic rats spontaneouslydevelop an inflammatory disease that shares manyfeatures with the human spondyloarthropathies,including sacroiliitis. Breeding the animals in agerm-free environment has allowed the disease to bedifferentiated into those features that require normalbacteria in the gut (i.e. the occurrence of diarrheafollowed by arthritis) and those that don’t (psoria-sis-like skin and nail lesions). The disease is pro-duced by T cells recognizing HLA-B27 expressed athigh levels on bone-marrow-derived antigen-pre-senting cells, and there is a critical requirement forbacterial components.

Family studies

The discovery of the remarkable association ofHLA-B27 with AS and related spondyloarthro-pathies was reported in 1973. It helped to revitalizethe clinical and genetic studies of these disorders,and also broadened our understanding of their widerclinical spectrum. However, we still do not knowexactly how HLA-B27 plays its role in diseasepredisposition.

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Familial occurrence of these diseases provides apotential clinical resource for uncovering all thegenes, other than HLA-B27, that make an indi-vidual susceptible to environmental (non-genetic)triggers that start the disease process. However,these environmental triggers are also not yet fullyknown.

Research studies of families where two or morefirst-degree relatives suffer from AS are currentlytaking place in North America and Europe. Thestudy involves obtaining blood samples frommembers of such families. The DNA from thesesamples is then analyzed in the research laboratoryin order to find all the genes that play a role indisease predisposition. Such genetic studies havenot as yet had an impact on clinical medicine, butonce we have a greater understanding of how genesinteract with the environmental agents that triggerdiseases, it will be possible to treat them more effec-tively and even prevent them.

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The spondyloarthropathies are related diseases thatinclude AS. The various forms of spondyloarthropa-thy usually begin in the late teens and early twen-ties, but they can also begin in childhood or later inlife. They show a strong association with HLA-B27,but the strength of this association varies markedly,not only between the various spondyloarthropathiesbut also among racial and ethnic groups.

The mode of presentation of spondyloarthropathiesis very varied and, with the exception of AS, may notnecessarily involve sacroiliitis or spondylitis. It mayalso not be always possible to differentiate clearlybetween the various forms of spondyloarthropathies,especially in their early stages, because they generallyshare many clinical features, both skeletal and extra-skeletal. However, this is not a serious clinicalproblem because it does not usually impact the treat-ment decisions.

Spondyloarthropathies other than AS include:

• the arthritis associated with chronic inflammatorybowel diseases (i.e. ulcerative colitis and Crohn’sdisease) or psoriasis (a chronic skin disease)

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• a form of juvenile chronic arthritis• reactive arthritis (Reiter’s syndrome)• undifferentiated forms of the disease.

Patients with psoriasis, ulcerative colitis, Crohn’sdisease, or reactive arthritis (Reiter’s syndrome) aremore likely to develop AS than the rest of thepopulation. The reverse is also true, i.e. patientswith AS are more likely than the general popula-tion to also suffer from Crohn’s disease, ulcerativecolitis, or psoriasis.

Doctors have found that the clinical featurestypical of spondyloarthropathies may occur in dif-ferent combinations, so the existing criteria fordisease classification may not be appropriate forsome patients. The European SpondyloarthropathyStudy Group (ESSG) have therefore developedclassification criteria (Table 5) to include this cur-rently recognised wider spectrum of spondylo-arthropathies.

Reactive arthritis (Reiter’ssyndrome)

Reactive arthritis is an aseptic inflammatory arthritisthat follows an episode of urethritis, cervicitis, ordiarrhea, and may also show inflammation at sitesother than joints, such as eyes, skin, and mouth. Thejoint inflammation is triggered by bacterial infectionat a distant site, usually in the gastrointestinal orgenitourinary tract.

Not everyone who develops these bacterialinfections will develop reactive arthritis. Somepeople are genetically susceptible and the inheri-tance of the HLA-B27 gene increases the risk of

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Table 5 The European Spondyloarthropathy StudyGroup (ESSG) criteria for classifying disease as aspondyloarthropathy

Spondyloarthropathy is defined as the presence of inflammatory spinalpain or synovitis and

one or more of the following:• family history: presence, in first- or second-degree relatives, of:

ankylosing spondylitis, psoriasis, acute iritis, reactive arthritis,or inflammatory bowel disease.

• psoriasis• inflammatory bowel disease• alternating buttock pain• enthesitis• acute diarrhea• urethritis• sacroiliitis: bilateral grade 2–4 or unilateral grade 3–4

Definitions used in these criteria

Inflammatory spinal pain: history of or current symptoms of spinalpain (low, mid and upper back, or neck region), with at least fourof the following five components:a at least 3 months durationb onset before age 45c insidious (gradual) onsetd improved by exercisee associated with morning spinal stiffness

Synovitis: past or present asymmetric arthritis, or arthritis predomi-nately in the lower limbs

Psoriasis: past or present psoriasis diagnosed by a doctor

Inflammatory bowel disease: past or present ulcerative colitis orCrohn’s disease diagnosed by a doctor and confirmed by radi-ographic examination or endoscopy

Alternating buttock pain: past or present pain alternating betweenthe two buttock regions

Enthesitis: past or present spontaneous pain or tenderness at exami-nation of the site of the insertion of the Achilles tendon orplantar fascia

Acute diarrhea: episode of diarrhea occurring within 1 monthbefore arthritis onset

Urethritis: nongonococcal urethritis or cervicitis occurring within1 month before arthritis onset

Sacroiliitis grading system: 0 = normal, 1 = possible, 2 = minimal, 3 = moderate, 4 = completely fused (ankylosed).

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reactive arthritis by about 50-fold. The diseasetends to be more severe and more likely to becomechronic in people with a triggering infection that issymptomatic and proven by bacterial culture, espe-cially if they are born with the HLA-B27 gene,than if the triggering infection produces no symp-toms and is suggested only by a positive antibodytest.

Depending on the bacterial trigger, reactivearthritis can be more common in men than inwomen. Table 6 lists some of the important bac-terial triggers. Genitourinary tract infection withChlamydia is the more commonly recognized initia-tor in the US, but enteric infections with Shigella,Salmonella, Yersinia, or Campylobacter are more com-mon triggers in developing countries. Sometimesthere is no recognized antecedent infection, or thetriggering infection may be asymptomatic. The termreactive arthritis is often used when the identity ofthe triggering organism is known, and it encom-passes the more restrictive and less commonly usedterm Reiter’s syndrome.

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Table 6 Bacteria triggering reactive arthritis

Chlamydia trachomatisShigella flexneriSalmonella (many species)Yersinia enterocolitica and Y. pseudotuberculosisCampylobacter fetus jejuniClostridium difficile

Note: Reactive arthritis not associated with HLA-B27 has also beenobserved following many bacterial, viral and parasitic infections, and inassociation with intestinal bypass surgery, acne, hidradenitis suppurative(abscesses in the armpit and groin), and cystic fibrosis.

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How common is reactive arthritis?

The prevalence of reactive arthritis in a populationvaries with that of HLA-B27 and the triggering bac-terial infections. Chlamydia-induced reactive arthri-tis is most commonly seen in young promiscuousmen. However, it is under-diagnosed in womenbecause their chlamydial infection is often subclini-cal or asymptomatic, and also because doctors rarelydo pelvic examinations to look for the presence ofcervicitis (inflammation of the cervix, the part ofthe uterus that protrudes into the vagina). Thepost-enteritic form of the disease affects childrenand adults, both male and female, including elderlypeople.

The incidence of Chlamydia-induced reactivearthritis has declined since 1985 in Europe and theUS, but the post-enteritic form of the disease maybe increasing. After some epidemics of bacterialgastroenteritis or food poisoning (e.g. Salmonellaenteritis) the incidence of reactive arthritis, or atleast some form of musculoskeletal inflammationand pain, can be as high as 20% among B27-positiveindividuals in the general population, but the initialepisode of reactive arthritis in such epidemics is rela-tively weakly associated with HLA-B27 (not morethan 33% of these patients may possess this gene).

To give one specific example, in the Finnishgeneral population aged 18–60 years the annualincidence of Chlamydia-induced reactive arthritis(confirmed by bacterial culture) is 4.6 per 100 000.The triggering genitourinary infection is asympto-matic in 36%. The annual incidence of post-enteritic reactive arthritis is 5 per 100 000; thetriggering enteric infection is asymptomatic in 26%.

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Symptoms of reactive arthritis

The clinical picture varies from mild arthritis to aseverely disabling illness that may render thepatient bedridden for a few weeks. Many peoplehave only one episode, but in some the disease doesrecur or persist. The arthritis more frequentlyinvolves the lower limbs, with the knees and anklesbeing most commonly affected, followed by the feet,the upper limbs, and the back. General symptomssuch as malaise, fever, and aching muscles (myalgia)may occur, and there may also be pain in the lowerback and the buttocks that feels worse in the earlymorning.

The acute arthritis is often associated with con-junctivitis or urethritis. Conjunctivitis (commonlyknown as pink eye) is an inflammation of the deli-cate outer membrane that lines the inside of theeyelids and the white of the eye. The inflammationis usually mild and bilateral, and you may not evennotice it. However, it can cause eye irritation andredness, and sometimes your eyelids may sticktogether in the morning. Some patients may getacute iritis (see Chapter 15).

Urethritis, an inflammation of the urethra (asmall tube through which urine passes from thebladder to the outside), can cause difficult or painfulurination. It occurs much more commonly in post-chlamydial reactive arthritis, and is more frequentlysymptomatic in men than in women, and maysometimes result in slight pus- or mucus-like ure-thral discharge, bladder inflammation (cystitis),lower abdomen pain, and urinary frequency.Sometimes the urethritis symptoms may be quitemild, and the doctor will have to ask about them.

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Prostatitis (an infection or inflammation of theprostate gland in men) often occurs in conjunctionwith urethritis. Women may develop cervicitis butoften there are no symptoms, and it may only bedetected by a pelvic examination.

People with post-enteritic reactive arthritis oftendescribe a history of fever, abdominal pain anddiarrhea, preceding the arthritis by 1–4 weeks. Theymay sometimes also have sterile (non-infected) ure-thritis.

Skin lesions can cause a lot of anxiety. A skinrash resembling psoriasis may appear on the soles ofthe feet and palms of the hands. These skin lesionsare called keratoderma blennorrhagica, and oftenheal within a few weeks but may need prescriptioncreams. In a few people small, shallow, painless soresmay occur on the tongue or roof of the mouth(palate), but they usually heal in a few days orweeks without any scarring, even without any treat-ment. Similar sores, called circinate balanitis,sometimes occur on the external genitalia – on thetip (glans) or shaft of the penis or on the scrotum inmen, and in the vagina in women. They crust overand heal after a few weeks. Finger- and/or toe-nailsmay show nail discoloration similar to that seen inpsoriasis, but without nail pitting or ridging.

Enthesitis is an important hallmark of reactivearthritis, and tendon sheaths and bursae may alsobecome inflamed. Sausage-like swelling of the toesor fingers may be a prominent finding in somepatients, just as in psoriatic arthritis. In the ankle,enthesitis can cause swelling, pain and tendernessin the back of the foot (Achilles tendinitis). Heelpain due to inflammation of the tendons, attached

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to the heel, which support, the arch of the foot(plantar fasciitis) is a frequent complaint.Ligamentous structures along the spine and sacroil-iac joints, and around the ankle and mid-foot, mayalso become inflamed. Psoriatic arthritis sharesmany features with reactive arthritis, and sometimesa long period of observation may be needed to reacha correct diagnosis.

Diagnosis

Diagnosis may sometimes be difficult, as there is nospecific diagnostic test. The erythrocyte sedimenta-tion rate (ESR) is often high, but this is common ininflammatory diseases. Other tests include examina-tion and cultures of synovial (joint) fluid, stool, andurethral discharge. A careful clinical history andphysical examination is needed to diagnose thiscondition. Because there is a delay of several daysbetween the triggering infection and the onset ofdisease, the patient may not relate the two eventsand therefore not mention the previous episode ofinfection to the doctor.

Outcome

In most people the disease can be well-managedwith treatment, and the outcome is usually goodbecause the disease is often self-limiting, i.e. it goesaway without any residual problems. Other peoplemay have recurrent attacks or have a chronic formof the disease with ongoing joint problems, typicallyrecurring arthritis and tendinitis that may result instiff joints and weak muscles.

Back and neck pain and stiffness due to sacro-iliitis and spondylitis may also occur. X-ray evi-

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dence of sacroiliac joint involvement is seen inabout 10% of patients during the acute phase, andmuch more frequently in chronic cases. Thespondylitis usually does not lead to the bamboospine typical of AS.

HLA-B27 is present in up to 70% of people withreactive arthritis, compared to 8% in the generalpopulation of Western European descent. The asso-ciation is weaker among some of the other races(e.g. only up to 40% of African–American reactivearthritis patients, and 2–3% of their general popula-tion possess B27). The presence of HLA-B27 canbe of some value as an aid to diagnosis in someappropriate clinical situations, but its absencecannot be used to exclude the diagnosis becausemany people with reactive arthritis do not haveHLA-B27. Patients who are B27-positive are morelikely to have back pain and stiffness, althoughsacroiliitis is often not visible on X-ray in earlystages. The disease is more likely to become chronicand evolve into spondylitis or be associated withacute iritis in people who are B27-positive.

Psoriatic arthritis

Psoriasis is a very common chronic skin disease,especially in populations of European extraction,and is present in up to 2% of the US population.There is abnormal proliferation of skin cells (calledkeratinocytes), induced by T lymphocytes, but theprecise cause is unknown. Psoriasis is usually seenin the form of itchy, dry, red, and scaly patches ofskin. Finger- and toenails may show discoloration(onycholysis), accompanied by pitting and ridging.

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An inflammatory arthritis occurs in more than10% of people with psoriasis. The arthritis precedesthe onset or the diagnosis of psoriasis in approxi-mately 15% of them. A family history of psoriasis orpsoriatic arthritis is present in up to 40% of peoplewith psoriatic arthritis, and family studies suggestthat several genes are involved (a multigenic modeof inheritance). Psoriasis is relatively much lesscommon in African-Americans, native Americans,and south-east Asians. The disease affects men andwomen equally and usually begins between30–50 years of age although it can begin inchildhood.

Sausage-like diffuse swelling of the toes or fingers(‘sausage digits’) may be a prominent finding insome patients, and enthesitis at bony sites of attach-ment of ligaments and tendons can cause painfulheels and a tender back. X-rays of the affected jointsmay show anything from mild erosion to severe bonedestruction and occasionally fusion of the joints.Psoriatic arthritis has been divided into five types:

• inflammatory arthritis, primarily involving thedistal small joints of fingers or toes

• asymmetric inflammatory arthritis, involving afew of the joints of the limbs

• symmetrical arthritis of multiple joints, resem-bling rheumatoid arthritis

• arthritis mutilans, a rare but very deforming anddestructive (mutilating) form

• arthritis of the sacroiliac joint and the spine(psoriatic spondylitis)

The exact prevalence of each of these forms ofarthritis is difficult to establish. Disease patterns

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may differ among various population groups, andmay even change with time in an individual. Somepatients may show overlapping features. Sacroiliitisoccurs in about 15% and predominant spondylitisin about 5%. Some people may get conjunctivitis oracute iritis. Spondylitis and acute iritis are morecommon in those who are B27-positive.

Enteropathic arthritis

Enteropathic arthritis develops in up to 20% ofpeople with Crohn’s disease or ulcerative colitis.This arthritis usually takes the form of peripheraljoint inflammation that correlates with flare-up ofthe bowel disease, especially in the case of ulcera-tive colitis, but one-fourth have axial disease (sacro-iliitis alone or with classic AS) that does notfluctuate with bowel disease activity.

Subclinical inflammatory lesions in the gut havebeen observed in spondyloarthropathy patientswithout gut symptoms. Follow-up studies suggestthat 15–25% of them will eventually develop clini-cally obvious Crohn’s disease, suggesting that theyinitially had a subclinical form of this disease.

Childhood (juvenile)spondyloarthropathies

Juvenile spondyloarthropathies are defined ashaving their onset before the age of 16. Recent datafrom pediatric rheumatology clinic registries inCanada, the UK, and the US indicate that approxi-mately 8% of all children referred to pediatricrheumatic disease clinics have a spondyloarthro-

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pathy, and among those children identified ashaving a discrete rheumatic disease, approximately20% suffer from spondyloarthropathy.

Improved guidelines for diagnosing childhoodrheumatic diseases have contributed to earlieridentification of childhood spondyloarthropathies.There is often no chronic inflammatory lower backpain, sacroiliitis, psoriatic skin lesions, or intestinalsymptoms, and as discussed later, undifferentiated forms of spondyloarthropathies occur more oftenduring childhood and adolescence than in adult-hood. Many patients may show a family history ofAS, psoriasis, inflammatory bowel disease, or acuteiritis. These spondyloarthropathies show a strongassociation with HLA-B27, just like AS of adultonset.

Intermittent episodes of pain in the groin, andresultant limping, without any previous physicaltrauma or infection, can be a presenting mani-festation in some children. Others may present withenthesitis at multiple sites. Some may present withthe syndrome of enthesits and arthritis (sometimescalled SEA syndrome, which stands for seronegativeenthesitis and arthritis). If the enthesitis affects the site of attachment of the patellar tendon to the tibial tubercle (a bony prominence an inch or so below the kneecap), it can sometimes be confused with a childhood condition calledOsgood–Schlatter’s disease. However, a child withjuvenile spondyloarthropathy will frequently alsoshow tenderness at other bony sites due to enthesi-tis, and not just at the tibial tubercles.

At least 50% of these young people reach adult-hood with persistent (active) arthritis and need

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further rheumatological care. Their disease mayevolve into juvenile-onset AS with back pain,sacroiliitis, and diminished spinal mobility. A studyof such patients in Mexico has found that severeenthesitis in the feet is a very common first presen-tation of AS in a Mestiso population of mixedgenetic ancestry (mostly native Americans withsome Spanish admixture).

Reactive arthritis including Reiter’s syndromecan also occur in children, usually triggered byenteric infection due to Shigella, Salmonella, orYersinia. There is an association with HLA-B27, but the arthritis is relatively less severe than inadults. The juvenile onset of psoriatic arthritis isuncommon but well documented.

Undifferentiatedspondyloarthropathy

The term ‘undifferentiated’ is used for a limitedform or early stage of the disease that does not meetthe criteria for AS, or the other spondyloarthro-pathies described above, which could be consideredas differentiated spondyloarthropathies. The un-differentiated forms can occur in adults too, but arerelatively more common in children. In fact, at least50% of spondyloarthropathies of childhood onsetpresent in an undifferentiated form.

The disease may begin with enthesitis causing painin the heels and other bony sites, or lower extremityarthritis of one (especially knee or ankle) or morejoints, mostly in boys between the ages of 9 and16 years, without any other features. This form ofarthritis may precede the back pain by several years.

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Less than 1 in 4 of children with AS or other dif-ferentiated spondyloarthropathies initially presentwith back pain, stiffness, or restricted motion, orsymptoms or signs of sacroiliitis. This is a notabledistinction from adults with AS. When a youngchild presents with isolated signs referable to thesacroiliac joint, possible bacterial infection of thesacroiliac joint is also considered. Sometimesleukemia and other forms of malignancy in childrenmay mimic the clinical presentation of juvenilearthritis, including spondyloarthropathy.

X-ray evidence of sacroiliitis is one of the diag-nostic hallmarks of spondyloarthropathies in theadult population. However, it is not easy to detectsacroiliitis by conventional radiography in grow-ing children. Dynamic MRI is helpful in childrenand adolescents with clinical features suggestive of a spondyloarthropathy, because it can dis-tinguish normal growth changes from true inflam-matory disease, and it does not involve exposure toradiation.

Treatment of spondyloarthropathiesother than AS

The initial treatment consists of the use of NSAIDsto treat joint problems. Persistent arthritis or enthe-sitis may require a corticosteroid injection into theaffected area. This is particularly helpful if the painand swelling continues to persist in one or morejoints. If the arthritis does not resolve within a fewweeks, additional medications such as sulfasalazine(Salazopyrin) or methotrexate (Rheumatrex) maybe needed. Anti-TNF therapy is very effective for

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patients with juvenile chronic arthritis resistant toconventional therapy.

Daily exercises stretching the joints involved (tokeep them from getting stiff), and muscle-strength-ening exercises (to regain strength and preventmuscle wasting and weakness) may be needed.Application of local heat or a warm shower maypromote relaxation and help in passive stretching oftight muscles. A heated swimming pool may alsohelp to decrease pain. When the acute phase of thearthritis resolves, low-impact exercises in the water(swimming and water aerobics) and stationary exer-cise bicycling can help improve exercise capability,muscle strength, and range of motion of the affectedjoint. Surgery can be helpful for people with severejoint damage.

Treatment of skin involvement in psoriaticarthritis

Psoriasis responds to topical corticosteroid medica-tions (ointments and creams), exposure to ultra-violet A light after application of photosensitizingpsoralene—the so called psoralen-photo-augmentedultraviolet A (PUVA) treatment, or treatment withvitamin D analogs. It is inadvisable to prescribe cor-ticosteroids by mouth to treat psoriasis because thishas untoward effects; in particular, the rapid taper-ing down of the dose can result in flare-up of skindisease. Refractory skin lesions may need metho-trexate or sulfasalazine. Cyclosporin has been usedwith good results, as has anti-TNF therapy,however, because of their side-effects and their highcost, these are only suitable for people with progres-sive disease unresponsive to other measures.

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Several studies have demonstrated that antibiotictreatment does not influence the course of reactivearthritis that has been triggered by enteric infection.It seems that once the enteritis trigger has beenpulled, the chain of events takes its path anyway.However, vigorous antibiotic treatment of Chlamydiare-infections has significantly reduced relapses ofreactive arthritis triggered by this organism.

Reactive arthritis itself is not contagious; onlythe triggering bacteria are. If the preceding infec-tion is transmitted sexually, as is the case with uro-genital chlamydial infection, it is advisable for thepatient’s sexual partners to be treated with anti-biotics at the same time. This helps to eradicate theinfection, or at least prevent it being transmitted toothers.

Use of sulfasalazine or methotrexate in peopleunresponsive to NSAIDs

Because of the efficacy of sulfasalazine in the treat-ment of inflammatory bowel disease and psoriasiseven in the absence of any associated arthritis, thisdrug may be especially useful for spondyloarthro-pathies associated with those diseases.

People with severe spondyloarthropathies withperipheral joint involvement who are unresponsiveto NSAIDs and sulfasalazine have sometimesresponded to weekly oral methotrexate (Rheu-matrex) therapy. Sometimes other immunosuppres-sants, such as azathioprine (Imuran), have beenused in the treatment of chronic inflammatoryarthritis resistant to conventional therapy. It isimportant to remember that sulfasalazine andimmunosuppressants are relatively slow-acting anti-

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rheumatic drugs, so patients should not expect aquick response. Moreover, these drugs, unlikeNSAIDs, are not pain relievers, although they canhelp relieve pain if they can first heal or control theunderlying inflammation that contributes to it.

Some patients with inflammatory bowel diseasemay need corticosteroid enemas or even oral corti-costeroids for control of severe flare-up con-sultations of the bowel disease, and also requireregular follow-up consultations with their gastro-enterologist. Treatment of severe chronic inflamma-tory bowel disease, specifically Crohn’s disease, withinfliximab (Remicade), is very effective, and mayalso control the associated arthritis and spondylitisquite well.

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International

The Ankylosing Spondylitis International Federation(ASIF) is a worldwide organization of national self-help societies for people suffering from AS or relateddiseases. It was established in 1988 to increase publicawareness and knowledge of these diseases around theworld and maintains a home page on the Internet:www.asif.rheumanet.org

The aims of ASIF are:

• exchange of information and experiences amongthe member societies

• cooperation in international research projects• exchange of articles for publication in the jour-

nals of the member societies• support of the development of newly formed

societies• establishment of contacts with AS patients in

countries where an AS society does not yet exist.

National and local

There are many such support groups and organiz-ations in various countries. Their aims are to:

Appendix 1Ankylosing spondylitisorganizations

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• contribute to the physical and mental health ofpatients with AS or related diseases

• organize supervised exercise and recreationaltherapy groups throughout each country

• arrange the exchange of experiences among thepatients

• oppose the social isolation of the patients• advise patients regarding social, medical and work-

related problems associated with their disease• cooperate with doctors and allied health

professionals• represent the interests of the patients in the

society, including the legislature (law) and thehealth services

• promote and encourage scientific research of thediseases

• increase public awareness and disseminateknowledge of the diseases in their respectiveregions or countries.These societies are listed below. However,

addresses (including homepage and e-mail addresses)and telephone and fax numbers do change from timeto time. An up-to-date list is maintained by ASIF ontheir Internet home page, www.asif.rheumanet.org

Useful information can also be obtained throughInternet home pages, such as www.spondylitis.org(based in the USA) and www.nass.co.uk (based inthe UK). These and many of the other supportgroups listed below enlist enthusiastic patient co-operation, and provide useful information, bookletsand pamphlets about AS and related spondylo-arthropathies for the people with AS and theirfamilies. Many of them can also provide adviceabout useful items such as wide-view mirrors for

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cars, working environment, insurance needs, jobs,exercises, and so on.

AustraliaAnkylosing Spondylitis Group of New South WalesPO Box 95, Artarmon, New South Wales 2064Tel. (0061) 2 9412 2505Email: [email protected]

Ankylosing Spondylitis Group of QueenslandPO Box 7366, East Brisbane, Queensland 4169 Tel. (0061) 7 3391 4689Email: [email protected] homepage: www.arthritis.org.au/asgroup

Ankylosing Spondylitis Group of Western Australia35 Wesley Street, Balcatta, Western Australia 6021 Tel. (0061) 9 344 5857

AustriaÖsterreichische Vereinigung Morbus Bechterew (ÖVMB)Obere Augartenstr. 26–28, A-1020 WienTel. (Mi 15–17h) and Fax: (0043) 1 33 22 810Email: [email protected] homepage: www.bechterew.at

Belgium (Flanders)Vlaamse Vereniging voor Bechterew-patiënten (VVB)c/o Leopold Bogaert, Pinksterbloemhof 16, 8300-Knokke-Heist Tel. (0032) 50 51 28 30Email: [email protected] homepage: www.vvb.rheumanet.org

CanadaAnkylosing Spondylitis Association of British Columbia (ASABC)2532 Western Avenue, North Vancouver, BC, Canada V7N 3 L1Email: [email protected]

Manitoba Ankylosing Spondylitis Associationc/o Lorne Ferley, 19 Carolyn Bay, Winnipeg, Manitoba, Canada R2J 2Z3, Tel. (001-204) 256–53 20, Fax (001-204) 231 19 12

Ontario Spondylitis Association (OSA)393 University Avenue, Suite 1700, Toronto, Ontario, Canada M5G 1E6

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Appendix 1: Ankylosing spondylitis organizations

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Tel. (001-416) 979-7228, Fax (001-416) 979-8366Email (Arthritis Society): [email protected] homepage of Arthritis Society: www.arthritis.ca

CroatiaHrvatsko drustvo za ankilozantni spondilitis (a section of Croatian Leagueagainst Rheumatism)Prof. Ivo Jajic, Vinogradska c. 29, HR-10000 Zagreb Tel. (00385-1) 37 87 248, Fax (00385-1) 37 69 067

Czech RepublicKlub bechterevikuc/o Revmatologicky ústav, Na Slupi 4, 128 50 Praha 2Tel./Fax (00420-2) 69 13 870Internet homepage: www.radio.cz/rhs/klubb

DenmarkLandsforeningen af Morbus Bechterew Patienterv/ Advokat Per Lignell, Rosenvænget 58, DK-8362 Hørning Tel. (0045) 86 92 33 00, Fax (0045) 86 92 30 65,Email: [email protected]

GermanyDeutsche Vereinigung Morbus Bechterew (DVMB)Metzgergasse 16, D-97421 SchweinfurtTel. (09721) 22033, Fax (09721) 22955Email: [email protected],Internet homepage: www.dvmb.rheumanet.org

Great Britain (UK)National Ankylosing Spondylitis Society (NASS)PO Box 179, Mayfield, East Sussex TN20 6ZLTel. (0044-1435) 873527, Fax (0044-1435) 873027Email: [email protected],Internet homepage: www.nass.co.uk

HungaryMozgáskorlátozottak Egyesületeinek Országos Szövetsége, Bechterew sectionc/o Dr.-Ing. Majtényi Sándor, Zrinyi utca 109/B, H-1196 Budapest Tel. + Fax (0036-1) 280 38 30

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IrelandAnkylosing Spondylitis Association of Ireland (ASAI)c/o Mr. Seoirse Smith, 6 Falcarragh Road, Gaeltacht Park, Whitehall,Dublin 9Tel. (+353-1) 83 76 614Email: [email protected]

ItalyAssociazione Italiana Spondiloartrite Anchilosante (A.I.Sp.A)c/o Favio Fornasari, Via Elisabetta Sirani, 3/2, I-40129 BolognaTel./Fax (0039-51) 37 23 23

JapanJapan Ankylosing Spondylitis Clubc/o Dr. Inoue Hisashi, 1-11-5, Shinkawa Mitaka-shi, Tokyo 181-0004Tel. (0081-422) 45-79 85, Fax (0081-422) 49-68 17

NetherlandsNederlandse bond van verenigingen van patiënten met reumatische aandoeningen, Commissie Morbus BechterewPostbus 1370, 3800 BJ AmersfoortTel. (0031-33) 61 63 64, Fax (0031-33) 65 12 00

NorwayNorsk Revmatikerforbund (NRF)/BekhterevPostboks 2653 Solli, N-0203 OsloTel. (0047) 22 55 72 16, Fax (0047) 22 43 12 51Email: [email protected],Internet homepage: www.rheuma.no

PortugalAssociação Nacional da Espondilite Anquilosante (ANEA)Rua Fernando Ribeiro, n: 57, P-2645-094 Alcabideche Tel. (00351-21) 46 02 511, Fax (00351-21) 46 02 509Email: [email protected] homepage: www.terravista.pt/mussulo/2553

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SloveniaDrustvo za ankilozirajoci spondilitis Slovenije (DASS)c/o Marjan Hudomalj, Parmova 53, SI-1000 Ljubljana Tel. (00386-61) 159 30 21, Fax (00386-61) 159 35 02Email: [email protected] homepage: www.drustvo-as.si

SpainCoordinadora Nacional de Espondilitis anquilosantec/o Liga Reumatológica Española (LIRE), C/ Cid 4-1º, Apartado 112,E-28001 MadridTel. (0034-1) 914358132 und (0034-1) 902113188Email: [email protected]ón Cordobesa de Enfermos Afectados de Espondilitis (ACEADE)Apartado de Correos 762, E-14080 Córdoba

SwedenBechterewreumatikernas Intresseorganisation (BERI)Seglaregatan 29 / Box 12031, S-402 41 Göteborg Tel. (Mo-Fr 10-15) (0046-31)147 147, Fax (0046-31) 122 305

SwitzerlandSchweizerische Vereinigung Morbus Bechterew (SVMB)Röntgenstr. 22, CH-8005 ZürichTel. (0041-1) 272 78 66, Fax (0041-1) 272 78 75Email: [email protected] homepage: www.bechterew.ch

SingaporeSingapore Ankylosing Spondylitis Club (SASC)c/o National Arthritis Foundation, 336 Smith Street, #06-302 NewBridge Centre, Singapur 050336Tel. (0065) 227-97 26, Fax (0065) 227-02 57Internet homepage: www.ttsh.gov.sg/medical/as/

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TaiwanAnkylosing Spondylitis Caring Society of R.O C.Dr Hwa-Chang Liu, Department of Orthopaedic Surgery, NationalTaiwan University Hospital, 7 Chung-Shan South Road, TaipeiTel. (00886-2) 397 08 00 Ext. 5688, Fax (00886-2) 395 69 88 Email: [email protected] homepages: www.health.nsysu.edu.tw/wei/as andwww.ascare.org.tw

UkraineSociety of Patients with Ankylosing Spondylitis (Bechterew’s disease),Bechterew group in Solotonoshac/o Osirskij Viktor Dmitrijewich), Boulevard Gagarina 13/8,Solotonosha, Ukraine 258100 Tel. (+380-475) 83 34, Fax (+380-475) 2172

USASpondylitis Association of America (SAA)PO Box 5872, Sherman Oaks, CA 91413Tel. (001-818) 981-1616, Fax (001-818) 981-9826Email: [email protected] homepage: www.spondylitis.org

Research organizationsAnyone interested in research studies in North America can contactthe following organizations:

Spondylitis Association of America (SAA)PO Box 5872, Sherman Oaks, CA 91413Tel. (001-818) 981-1616, Fax (001-818) 981-9826Email: [email protected],Internet homepage: www.spondylitis.org

North American Spondylitis Consortium (NASC)Internet homepage: www.asresearch.org

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In Europe research studies into AS have also been under way for someyears. Interested families there can contact the following organizations:Wellcome Trust Centre for Human Genetics, Oxford, EnglandConsortium Européen pour les études génétiques et immunogénétiquesde la Spondalarthrite Ankylosante et des autres Spondylarthropathieswith research partners in Belgium, Finland, France, Germany, GreatBritain, Italy, Portugal, and Sweden.

Internet-based rheumatologyeducationwww.rheuma21st.comhttp://rheuma.bham.ac.ukwww.jointandbone.orgwww.nlm.nih.gov/medlineplus

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Achilles tendinitis inflammation of the Achillestendon, causing swelling and tenderness at the lowerend of the calf where it inserts into the heel bone

acupuncture an ancient medical procedure that origi-nated in China more than 2000 years ago. It is basedon the theoretical concept of balanced Qi (pronounced‘chee’) or vital energy that flows throughout the bodyvia certain pathways that are accessed by puncturingthe skin with hair-thin needles at particular locationscalled acupuncture points. Stimulation of acupuncturepoints is believed to stimulate the brain and spinal cordto release chemicals that change the experience of painor cause biochemical changes that may stimulatehealing and promote general well-being. See also alter-native and complementary remedies; traditionalChinese medicine

allele alternate forms of a gene at a distinct location(locus) on a chromosome

alternative and complementary remedies these includeholistic medicine, folk remedies, and alternative thera-pies (herbal medications or extracts, homeopathy,Ayurvedics, and traditional Chinese medicine (TCM).These complementary and alternative treatments aremostly based on anecdotal evidence, primarily from

Appendix 2Glossary

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individuals who report their own successful use of thetreatment. One needs to apply scientific methods toestablish the validity of the anecdotal evidence

amino acids small organic molecules which are thebuilding blocks of peptides and proteins

amyloid a proteinaceous fibrillar material deposited invarious tissues and organs, sometimes secondary to achronic inflammatory disease

analgesia pain relief, e.g. by such drugs as paracetamol,NSAIDs or narcotics. These pain-relieving drugs arecalled analgesics (see NSADs)

ankylosing hyperostosis also called Forestier’s diseaseor diffuse idiopathic skeletal hyperostosis (DISH). Itcauses excessive new bone formation along the spineand other sites (at entheses), can result in stiff spinethat may be confused with AS

ankylosing spondylitis (AS) an inflammatory arthriticdisorder, primarily of the axial skeleton (sacroiliacjoints and spine), but can affect hip and shoulder jointsand infrequently the peripheral joints. It causes chronicback pain and leads to stiffness of the spine. Most ofthe affected individuals have the HLA-B27 gene

ankylosis fusion, which may be fibrous, or bony (as inAS)

annulus fibrosus the tough outer fibrous layer of theintervertebral disc

antibodies proteins produced by white blood cells(plasma cells and B lymphocytes) that confer immunity

antigen a substance that causes the body’s immunesystem to produce antibodies that try to eliminate itbecause the body sees the antigen as foreign or harmfulsubstance (e.g. from invading viruses or bacteria)

antigen-presenting cell a cell that ingests and processesforeign substance (e.g. from invading viruses or bac-teria) and displays the resulting antigen fragments(small peptides) on its surface to activate those T cellsthat respond specifically to that antigen

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aortitis inflammation of the aorta, which is the mainartery that carries the blood from the heart to ulti-mately supply the needs of the body

arachnoiditis fibrosis (scarring) of the membrane cov-ering the spinal cord and spinal nerve roots as theypass through the spinal canal. This results in entrap-ment of these nerve roots that may cause chronic backand leg pain and neurological dysfunction. It canoccur following spinal surgery, and has also been asso-ciated in the past with the use of an X-ray contrastmedium. Very rarely it can occur in the lower end ofthe spinal canal in AS without any apparent reason,and is the cause of cauda equina syndrome in thisdisease

arthralgia pain in one or more joints without anyoutward evidence of a joint abnormality

arthritis literally means inflammation of the joint, andis used to refer to more than 100 joint diseases, some ofwhich may also affect other regions of the body. Theplural is arthritides

arthritis mutilans an extremely destructive form ofarthritis; the term is usually applied to a very severeform of psoriatic arthritis

arthrocentesis taking a sample of joint fluid out fortesting, obtained by a needle puncture of the joint.Sometimes all of the joint fluid may be aspirated as apart of treatment

arthrodesis a surgically induced or spontaneous fusionof a joint

arthroplasty surgical procedure to alter a joint, e.g. itsexcision and replacement by an artificial joint

arthroscopy inspection of the inside of a joint, e.g. forobtaining a biopsy, usually through a fiber optic instru-ment called arthroscope

autoimmune disease a disease in which the immunesystem attacks and destroys the body’s own tissues thatit mistakenly believes to be foreign

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axial arthritis arthritis in the spine and/or neighboringjoints, especially the sacroiliac joints, as in AS, in con-trast to arthritis of peripheral (limb) joints

Ayurveda the traditional Indian medical system, whichclaims that health is based on a harmonious relation-ship between three humors called ‘doshas’, and dishar-mony results in disease

B cells (B lymphocytes) antibody-producing whiteblood cells, which mature in the bone marrow. Theletter B originally came from bursa of Fabricius where Blymphocytes originate in chickens, but has subse-quently been extended to imply the bone marrow

bamboo spine X-ray appearance of spine in advancedAS because of spinal fusion producing a bamboo-likeappearance

biologic response modifiers drugs which are also calledbiologicals for short, and include anti-TNF drugsinfliximab (Remicade) and etanercept (Enbrel)

biopsy removal of a small tissue specimen for examina-tion

bisphosphonates drugs used to treat osteoporosisbecause they inhibit bone resorption

bowel a word commonly used for the small and largeintestines (see gut; large intestine; small intestine)

brand name the brand name (trademark) of a drug iscoined by the manufacturer in agreement with theregulating agencies, unlike the generic name whichindicates its active ingredients. For example, cele-coxib is the generic name for the drug whose brandname is Celebrex. The brand name starts with acapital letter but the generic name does not. Severalbrand name drugs can have the same generic name ifthey contain the same active ingredient. Thus,Motrin and Aleve are both brand names for thegeneric drug ibuprofen

bursa a fluid-filled sac found between tissue planes overbony places subject to shearing forces, as over the

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elbow and knee. It is lined by synovium that secretesthe lubricating fluid

bursitis an inflammation of a bursacalcification deposition of chalky (calcific) material in

tissue, leading to bone formationCampylobacter a type of bacteria. Enteric infections

with these bacteria can trigger reactive arthritis insusceptible individuals

capsule a thick membrane joining together ends of twoadjacent bones to form a joint. Its inside is lined withsynovium that forms the joint fluid

cartilage a tissue that covers the ends of bones to forma smooth shock-absorbing surface for joints, and resultsin very low friction movement. Cartilage also occurs atother sites, such as the nose and the ears

cauda equina syndrome some people with advancedAS on rare occasions may get this neurologicalcondition resulting from gradual scarring at the lowerend of the spine that entraps the lower spinal nerves.The name cauda equina means horsetail, so namedbecause the lowermost spinal nerves slope downward asa bunch before they exit the vertebral column.

CD4+ (CD8+) T lymphocytes these T cells carry amarker on the surface known as a cluster of differentia-tion (CD) marker which can be either CD4 or CD8.The CD4+ T cells, also known as helper T cells, helporchestrate the antibody responses, and the CD8+ Tcells—also called cytotoxic (destructive to cells) orsuppressor T cells—are involved in cell-mediatedimmunity that targets infected cells

celiac disease inability to digest and absorb a proteinfound in wheat, resulting in poor absorption of nutri-ents from the foods because of damage to the lining ofthe small intestine; also called gluten intolerance ornon-tropical sprue

cervicitis inflammation of the cervix, the part of theuterus that protrudes into the vagina

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chromosome a thread-like structure within the nucleusof a cell that contains the genes. There are 46 chromo-somes in the nucleus of a human cell; 22 of them are inpairs that are given the numbers 1–22, and the remain-ing two are the X or Y chromosomes (sex chromo-somes) that determine a person’s sex—males have oneX and one Y chromosomes, and females have two Xchromosomes

Chlamydia trachomatis a bacterium that has apredilection to infect the genitourinary tract. Such aninfection is the more commonly recognized initiator ofreactive arthritis in the US

Clostridium difficile bacteria that are normally presentin the large intestine, can cause a serious illness calledpseudo-membranous colitis in people taking anti-biotics, and can sometimes trigger reactive arthritis

collagen and connective tissue a set of fibrous proteinsand supporting framework that form the main buildingblocks of the body, including the internal organs, liga-ments, tendon, cartilage, bone, and skin

conjunctivitis commonly known as pink eye; it is aninflammation of the delicate outer membrane that linesthe inside of the eyelids and the white of the eye

contracture arthritis or prolonged immobility can resultin the involved joint becoming less freely moveable.Associated with shortening and wasting of muscles

control group in clinical studies the control group,which is given either the standard treatment for amedical condition under study or an inactive substance(called a placebo), is compared with a group given anexperimental treatment to find its efficacy for thedisease under study

coping the psychological processes following any stress-ful situation

cortisone a natural hormone made by the adrenalgland. Sometimes wrongly used as a synonym for corti-costeroids

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corticosteroids a group of related compounds which,like cortisone, reduce inflammation and irritationcaused by many disease processes, including manyforms of arthritis, and skin and bowel diseases

Crohn’s disease a chronic inflammatory bowel disease(also called ileitis or regional enteritis), that can affectthe entire gastrointestinal tract, though it usuallyinvolves the lower small intestine, (the ileum) and theadjacent part of the colon

C-reactive protein (CRP) its measurement in theblood can be used to detect or grade inflammation

cytokine a soluble protein, produced by white bloodcells, that acts as a messenger between cells, eitherstimulating or inhibiting the activity of various cells ofthe immune system. There is normally a very delicatebalance among the various cytokines

cytoplasm a liquid compartment in the cell, sur-rounding the central nucleus. The cytoplasm containsmitochondria and other structures or componentsresponsible for normal protein formation, secretion andother cell functions

DEXA bone scan a means of measuring the bonedensity to detect osteoporosis at a much earlier stage ascompared to a standard X-ray. DEXA stands for dual-energy X-ray absorption i.e. X-ray absorption at twodifferent quantum energies or wavelengths

disorder a synonym for diseasedisability in the context of health experience, a dis-

ability is a restriction or lack (resulting from an impair-ment) of ability to perform an activity in the manner orwithin the range considered normal (see WHO, 1980)

distal farther away from the trunk. For example, a handis the distal end of an arm. The opposite is proximal

DNA a double-stranded, helical molecule that carriesgenetic information, primarily present within the nucleusof each cell in plants and animals. It tells the cellsexactly what to do and how to perform their functions

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double-blinded a doubled-blinded trial produces moreobjective and unbiased results because neither theresearch investigators nor the study participants knowwho is receiving the investigational drug and who isreceiving the placebo

duodenum the first part of the small intestine. An ulceron its inner lining is called a duodenal ulcer

dowager’s hump hump in the upper back (thoracickyphosis) in an elderly woman with osteoporosis

dysentery an infectious disease of the intestine thatcauses bloody, mucus-filled diarrhea, which can beaccompanied by abdominal pain or cramps, fever, anddehydration from excessive diarrhea. It is caused byenteric infections, usually with Shigella, and can some-times trigger reactive arthritis

elimination diet requirement that certain foods shouldnot be eaten

enteritis an inflammation (irritation) of the smallintestine

enthesis site of attachment of ligament or tendon tobone

enthesitis inflammation of an enthesisenthesopathy an all-inclusive term that covers all

abnormalities of an enthesis (e.g. enthesitis is aninflammatory type of enthesopathy)

enzyme a protein that acts to promote or facilitatecertain biochemical processes in the body; e.g. manyenzymes produced in the gut assist in digestion of food

epicondylitis enthesopathy at bony prominence (epi-condyle) of the elbow; may occur on the medial(inner) side (golfer’s elbow) or the lateral (outer) side(tennis elbow)

erythrocyte sedimentation rate (ESR) a blood testcommonly used to detect or grade inflammation

esophagus the tube-like passage through which swal-lowed food travels from the mouth to the stomach

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familial a term used to indicate a disease or a trait (aninherited characteristic) which tends to affect morethan one member in a family

fascia tough membrane that encloses muscles and otherorgans

fasciitis inflammation of the fascia.fibromyalgia a complex chronic painful condition, pri-

marily occurring in women, characterized by widespreadmusculoskeletal pain, and fatigue, and accompanied bytender points at defined locations, often associated witha non-restorative sleep pattern

fibrositis a term used interchangeably with fibro-myalgia

folic acid and folinic acid members of the vitamin Bcomplex

food poisoning an acute food-borne gastrointestinalinfection caused by food contaminated by harmfulbacteria that results in symptoms such as diarrhea,abdominal discomfort or cramps, and fever

Forestier’s disease see ankylosing hyperostosisgastric ulcer an ulcer on the inner lining of the

stomachgastrointestinal tract alimentary tract, including

esophagus, stomach, duodenum, ileum, large bowel,and rectum

gene part of the DNA molecule responsible for makingproteins. It is the basic unit of heredity; all informationin the genes (genetic information) is passed fromparent to child

generic name see brand namegenetic counseling informing people about genetic

facts that may guide them in making a decision basedon a knowledge of disease risk. The word genetic refersto any characteristic that is inherited

genetic marker a gene that is used to identify an indi-vidual disease or trait, or trace its inheritance within afamily

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genitourinary tract the genitalia, the bladder, and theurethral tube through which the bladder empties

gut a word in common use to describe the large andsmall intestine (see bowel, large intestine; smallintestine)

handicap in the context of health experience, a handi-cap is a disadvantage for a given individual, resultingfrom an impairment or a disability, that limits or pre-vents the fulfillment of a role that is normal (depend-ing on age, sex, and social and cultural factors) for thatindividual (see WHO, 1980)

H2-blockers medicines such as cimetidine (Tagamet),ranitidine (Zantac), or famotidine (Pepcid), used totreat acid indigestion, heartburn, and ulcer pain. Theyare so called because they act by blocking histamine-2signals to reduce the amount of acid produced by thestomach

heartburn symptoms caused by stomach acid flowingback into the esophagus

Helicobacter pylori a corkscrew-shaped bacteriumfound in the stomach that can predispose to stomachand duodenal ulcers. Previously called Campylobacterpylori

heterozygote and homoozygote an individual inherits aset of two alleles for each HLA locus from his or herparents. For instance, an individual may inherit HLA-B27 from one parent and HLA-B8 from the other.Most individuals do not inherit the same gene (belong-ing to a locus) from both parents, and are said to beheterozygotes. Someone who inherits the same gene,e.g. HLA-B27, from both parents is homozygous forHLA-B27

HLA human leucocyte antigens. These are cell surfaceproteins, detected by blood testing, that vary fromperson to person. They are also called tissue antigens orhistocompatibility antigens because ideally organdonors and recipients must have compatible HLA;

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otherwise the transplanted organ is recognized as non-self (‘foreign’) and is rejected. HLA are related to theworkings of the immune system; they present self- andforeign-derived (e.g. viral) peptides (a few amino acidslinked together) to T lymphocytes and other cells ofthe immune system that help the body fight illness.They are of two broad types, called class I and class IIHLA. Their genes are located on chromosome 6; theloci are given the letters A, B, C, D, and so on

HLA-B27 an HLA class I molecule that has beenassigned the number 27; its gene is present at the Blocus. There are quite a few HLA antigens that confersusceptibility to certain diseases: HLA-B27 to AS, andHLA-DR4 to rheumatoid arthritis, for example

hydrotherapy physiotherapy in a pool (usually heated)idiopathic of unknown cause or explanationileum the major part of the small intestine (see small

intestine)ilium (or iliac bone) major bony component of the

pelvis. There is one on each side, joined to the sacrumvia the right and left sacroiliac joints

impairment in the context of health experience, animpairment is any loss or abnormality of psychological,physiological or anatomical structure or function(WHO, 1980)

incidence the rate of occurrence of some event, such asthe number of individuals who get a disease divided bya total given population, per unit of time (usually peryear). In contrast to prevalence, incidence describesthe number of new cases of a disease among a certaingroup of people for a certain period of time, i.e. howoften a new case is diagnosed

inflammation a typical reaction of tissues to injury ordisease, usually marked by four signs: pain, swelling,redness, and heat. It may be acute (as in a burn or ingouty arthritis) or chronic (as in rheumatoid arthritisor chronic infections such as tuberculosis)

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inflammatory bowel disease a chronic (long-lasting)inflammatory disease of the gut, e.g. ulcerative colitisor Crohn’s disease

internist a doctor specializing in internal medicine(not requiring surgery).

intestine also called bowel or gut (see large intestine;small intestine)

intestinal flora bacteria and other organisms thatnormally grow in the intestine

intestinal mucosa surface lining of the intestines wherethe absorption of nutrients takes place.

intra-articular into or within a joint, e.g. intra-articularinjection

joint the place where two bones meet. Most joints arecomposed of cartilage, joint space, fibrous capsule, jointlining (synovium) and ligaments

juvenile chronic arthritis arthritis in children 16 yearsof age or less, that has been present for at least3 months, and for which no other cause is obvious. It isnow preferably called juvenile idiopathic arthritis

keratoderma blennorrhagica rash on palms of thehands and soles of the feet which may occur in reactivearthritis (Reiter’s syndrome); it resembles a form ofpsoriasis

kyphosis forward stooping (bowing) of the spine(‘humpback’ deformity)

large intestine part of the intestine that changes stoolfrom a liquid to a solid form by absorbing water. Oftensimply called the colon, but in fact includes the appen-dix, cecum, colon, and rectum; has a total length ofabout 5 feet (1.5 m).

leukocyte white blood cell, part of the immune systemligament stretchy tough band of cord-like tissue that

connects bones together, and confers stability byrestraining excessive joint movement

limb girdle joints hip and shoulder jointslocus precise location of a gene on a chromosome

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lymphocyte a type of white blood cell present in theblood, lymph and lymphoid tissues; primarily responsi-ble for immune responses (see also B lymphocytes;CD4+ (CD8+) T lymphocytes; T lymphocytes)

macrophage a relatively large immune system cell thatdevours invading bacteria and other intruders, andstimulates other immune cells by presenting them withsmall pieces of the invaders. Can sometimes harborlarge quantities of invading viruses like HIV withoutbeing killed, and thus act as a reservoir of such viruses

magnetic resonance imaging (MRI) a method oftaking pictures of the soft tissues in the body that areclearer than those obtained by X-rays, and withoutradiation

medial on the inside (as opposed to lateral); not to beconfused with the median nerve which is compressedin carpal tunnel syndrome

methotrexate a drug which is used in low doses for thetreatment of inflammatory disorders, including varioustypes of inflammatory arthritis, and in very high dosesto treat certain cancers. It is sometimes abbreviated toMTX (See also slow-acting anti-rheumatic drugs)

monoclonal antibodies artificially produced antibodiesused in research and also for treatment of somediseases. They are produced in a cell culture (clone) bymultiplying one single mother cell thus having exactlythe same properties (very pure antibody)

MRI See magnetic resonance imagingnucleus the central controlling structure within a

living cell that contains the genetic codes (in chromo-somes) for maintaining life systems of the cell and forissuing commands for cell growth and reproduction

nausea the feeling of wanting to throw up (vomit)neurohormones biochemical substances made by tissue

in the body’s nervous system that can change the func-tion or structure, or direct the activity of tissues ororgans; e.g. neurotransmitters

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neurological relating to the body’s nervous system,which oversees and controls all body functions

neurotransmitters biochemical substances that stimu-late or inhibit nerve impulses in the brain that relayinformation about external stimuli and sensations, suchas pain

NK (natural killer) cells non-specific lymphocytes likekiller T cells that attack and kill cancer and infectedcells. They are natural killers because they do not needto recognize a specific antigen in order to attack andkill

NSAID (non-steroidal anti-inflammatory drug) non-cortisone, non-addictive (non-narcotic) drug thatreduces pain and inflammation and is therefore used inthe treatment of pain and arthritis

oligoarthritis inflammation of up to four joints; if morejoints are involved, then the disease is called poly-arthritis

onycholysis nail abnormality and discoloration seen inpsoriasis and reactive arthritis; may be accompanied bypitting of the nail in psoriasis.

Osgood–Schlatter’s disease a childhood condition ofthe site of attachment of the patellar (kneecap) tendoninto the tibial tubercle, a bony prominence an inch orso below the kneecap. Results in localized pain andtenderness that can sometimes be confused with enthe-sitis at this site seen in some children with juvenile ASand related diseases

osteitis condensans ilii increased bone density (sclero-sis) at the sacral side of the sacroiliac joint that is ofunknown cause and is usually without symptoms. Its X-ray appearance can be confused with sacroiliitis

osteoarthritis (osteoarthrosis) degenerative disorder ofjoints, most often from disease in the spine and in theweight bearing joints (knees and hips). Normally seenwith aging, but can occur prematurely due to variousreasons, for instance after an injury to a joint. Also

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known as degenerative joint disease, it can cause jointpain, loss of function, reduced joint motion, anddeformity

osteomalacia bone-thinning disorder resulting fromdeficiency of vitamin D. Can be mistaken for osteo-porosis, and can also be confused with spondylitis. Thechildhood form of osteomalacia is called rickets

osteophyte bony outgrowth (seen on X-ray) at jointmargin of an osteoarthritic joint, or in degenerativedisc disease

osteoporosis a disease characterized by reduction inmineral content usually seen with aging, but also inconnection with certain conditions such as paralysis, ordue to prolonged use of certain drugs, such as corti-costeroids

Paget’s disease a disease characterized by acceleratedbone turnover, resulting in the involved bone becom-ing enlarged but weak and fragile. The bone also feelswarmer to touch due to increased blood supply. Alsocalled osteitis deformans

pathogenesis process of development of a diseasepauciarthritis same as oligoarthritispelvis the bony structures in the lowest part of the

trunk. The term pelvic is used for anything thatbelongs or refers to the pelvis

peptic ulcer a sore in the lining of the stomach (gastriculcer) or duodenum (duodenal ulcer). The wordpeptic refers to the stomach and the duodenum, wherepepsin is present, an enzyme that breaks down proteins.An ulcer can sometimes occur in the lower part of theesophagus in association with heartburn.

peptide a few amino acids linked together. Proteins aremade of multiple peptides linked together

placebo originally a Latin word meaning ‘I will please’.Now used for inactive substance (sham) given to par-ticipants of a research study in order to test theefficacy of another substance or treatment. In short-

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term clinical trials, many of the most valued drugs inclinical use are only about 25% more effective thanplacebo. Scientists often have to compare the effectsof active and inactive substances to learn more abouthow the active substance affects participants; in suchstudies both doctor and patient are unaware of who isreceiving the active or inactive substance. Suchstudies are known as double blind placebo controlledstudies

polyarthralgia pains in many joints; conventionallyrefers to more than four joints, without signs ofinflammation in the symptomatic joints

polyarthritis inflammation in many joints; convention-ally in more than four joints

preclinical diagnosis diagnosis of a genetic diseasebefore there are any symptoms or signs

prevalence the observed number of people in a givenpopulation affected with a particular disease or con-dition at a given time, usually stated as the number ofcases observed per 100 000 individuals, or listed as apercentage. In contrast with incidence, prevalence canbe thought of as a snapshot of all existing cases at aspecified time

prognosis the probable end result or outcome of adisease

protein a large molecule composed of amino acids.Essential components of the body tissue (see alsopeptide)

proton pump inhibitors a group of drugs used to treatheartburn and peptic ulcer disease. These includeomprezole (Prilosec), esomeprazole (Nexium) andpansoprazole (Prevacid)

prospective, randomized, double blind study clinicaltrial or study in which the method of data analysis isspecified in a protocol before the study is begun(prospective). Patients are randomly assigned toreceive either the study drug or an alternative treat-

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ment, and neither the patient nor the doctor conduct-ing the study knows which treatment is being given towhich patient (see also placebo)

proximal the part of a limb that is closest to the trunk.For example, the shoulder joint forms the proximal endof the upper extremity (opposite of distal)

psoriasis a common chronic skin disease, morecommon in whites (2% of the population) than inother racial groups, in which red flaky lesions occur—often on the elbows and knees, or in the scalp. Maycause nail abnormalities

psoriatic arthritis arthritis associated with psoriasis;occurs in more than 10% of people with psoriasis. Mayoccur in several forms

Qi Chinese term for vital energy or life force.Pronounced chee (see acupuncture)

radiography/radiograph/radiogram/radiologic radiogra-phy (or roentgenography) is the method of taking apicture with the help of X-rays, and the terms radi-ograph or simply X-ray are sometimes used for theresulting picture. Radiogram is the correct name for animage taken by radiography

randomized, double-blind, placebo-controlled, multi-center trial a clinical trial in which patients havebeen randomly assigned to receive either the study drugor the alternative treatment under study. Neither thepatient nor the doctor conducting the study knowswhich treatment is being given; the alternative to thestudy drug is a placebo; and the study is conducted atseveral centers

range of motion the extent to which a joint is able togo through all of its normal movements. Range-of-motion exercises help increase or maintain flexibilityand movement in muscles, tendons, ligaments, andjoints

reactive arthritis arthritis resulting from infection else-where in the body; i.e. there is no infection in the

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joint. The commonest type is HLA B27-related andmay follow certain types of bowel or genitourinaryinfection

Reiter’s syndrome a form of HLA B27-related reactivearthritis with a classical triad of arthritis, conjunctivi-tis, and urethritis, with or without other features ofspondyloarthropathies. The term reactive arthritis isnow used more commonly to describe this condition

rheumatic fever a form of reactive arthritis triggered bystreptococcal sore throat. Its features include verypainful joint inflammation (arthritis). It is now uncom-mon in developed countries but still occurs commonlyin other parts of the world. It can cause inflammationand scarring of heart valvos (rheumatic heart disease)

rheumatoid arthritis a chronic systemic disease thatcauses inflammatory changes in the synovium, or jointlining, that result in pain, stiffness, swelling, and ulti-mately loss of function and deformities of the affectedjoints due to destruction of the cartilage and adjacentbone. The disease can also affect other parts of thebody. In the past it was also called chronic polyarthri-tis. It is more common in women than men, and atleast 70% of patients show a positive blood test forrheumatoid factor

rheumatologist a doctor (board-certified internist orpediatrician) who has had specialized training in diag-nosing and treating disorders that affect the joints,muscles, tendons, ligaments, connective tissue, andbones

roentgenography see radiographysacroiliac joints two joints, one on either side, in the

lower back, between the two pelvic bones calledsacrum and ilium (see Figure 4)

sacroiliitis inflammation of the sacroiliac joint; bi-lateral sacroiliitis is a hallmark of AS

sacrum major bony component of the pelvis, shapedlike a wedge on which the spine rests. It forms a joint

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with ilium, one on each side, via the right and leftsacroiliac joints

Salmonella a group of bacteria comprising many dif-ferent types that may cause intestinal infection anddiarrhea called salmonellosis, which includes typhoidfever. Enteric infections with Salmonella, Shigella,Yersinia, or Campylobacter are the most common trig-gers for reactive arthritis, especially in some developingparts of the world

SAPHO syndrome so named because of its salient fea-tures: synovitis, acne, palmoplantar pustulosis, hyper-ostosis, aseptic osteomyelitis. This rare disease causesaseptic (no evidence of infection) bone necrosis atmultiple sites that can include the sacroiliac joints orthe spine. It is known by many different names, butSAPHO syndrome is the most common

sausage digit finger or toe that is diffusely swollen as aresult of tenosynovitis; usually seen in psoriatic andreactive arthritis. It is also called dactylitis

Scheuermann’s disease a non-inflammatory spinaldisease that occurs in adolescence and affects thethoracic spine, especially the discs. Often painless, butcan result in a stooped back

Schober’s test to detect the ability to bend forward(flexibility) of the lumbar spine (see Figure 5g andaccompanying caption)

scoliosis a non-inflammatory rotational deformity ofthe spine; results in a lateral curvature

selective estrogen receptor modulators (SERM) aclass of drugs used in the treatment of osteoporosis;they mimic the effect of estrogen but in a tissue-selective manner

septic arthritis bacterial infection of one or morejoints; requires urgent diagnosis and treatment

seronegative arthritis an arthritis that is not associ-ated with the presence of an autoantibody calledrheumatoid factor in the blood. Most people with AS

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and related spondyloarthopathies lack this auto-antibody, and therefore these diseases are examples ofseronegative arthritis. On the other hand, only about25% of people with rheumatoid arthritis are sero-negative

Shigella a group of bacteria that can cause an illnesscalled shigellosis, with high fever and acute diarrhea,sometimes mixed with blood (dysentery). Entericinfections with Shigella can trigger reactive arthritis

sibling brother or sisterskeletal muscles muscles that move the bony skeleton,

i.e. provide movement at the jointsslit lamp an instrument used by eye specialists (opthal-

mologists) to look for inflammation or other diseasesinside the eye

slow-acting and symptom-modifying anti-rheumaticdrugs (SAARDS and SMARDs) drugs such as sul-fasalazine and methotrexate, which may be useful inspondyloarthropathies that are resistant to conven-tional therapy. Any benefit from these drugs takes sometime to manifest itself, hence the name. UnlikeNSAIDs, these drugs are not pain relievers, but theywill help relieve pain if they can first heal or controlthe underlying inflammation

small intestine the tubular organ, about 20 feet (6 m)long, where most digestion occurs. It is made up ofthree parts: the duodenum (which is attached to thestomach), jejunum, and ileum (which ends in thelarge intestine)

spondylitis literally means inflammation of the spine,and is best exemplified by ankylosing spondylitis (AS)

spondyloarthritis and spondyloarthopathy AS andrelated diseases are grouped under this term. Thesediseases show clinical similarities to some extent, andoccur much more often in people who carry the HLA-B27 gene

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spondylolisthesis a loss of spinal column alignmentthat results from one vertebra slipping forward on topof another

spondylosis non-inflammatory degenerative (wear andtear) disease of the spinal column as we get older, suchas degenerative disc disease

steroids see corticosteroidsstomach ulcer an open sore in the lining of the

stomach. also called gastric ulcersulfasalazine see slow-acting anti-rheumatic drugssyndesmophytes ligamentous bone deposits (ossifica-

tion) producing fine bony bridging between adjacentvertebral bodies at the margin of the vertebrae, charac-teristic of AS. They are vertically orientated, unlikeosteophytes (seen in degenerative disc disease), whichgrow horizontally

syndrome a complex of signs and symptoms that whenoccurring together suggest a particular disease

synovium a thin membrane (normally one or two celllayers thick) lining the inside of the joint capsule. Itproduces synovial fluid for lubrication and nourish-ment of the joint cartilage

synovitis inflammation of the joints resulting frominflamed synovium; this results in joint inflammation(arthritis)

T cell (or T lymphocyte) T stands for the thymus,where T lymphocytes mature. T cells are white bloodcells that play a critical role in immune response, but,unlike B lymphocytes, do not produce antibodies(immunoglobulins). There are two main subtypes: theCD4+ helper T cells and the CD8+ cytotoxic orsuppressor T cells

Tai Chi a traditional Chinese mind–body relaxationexercise consisting of 108 intricate exercise sequencesperformed in a slow relaxed manner over a 30 minuteperiod

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temporo-mandibular joint (TMJ) the jaw jointtendon a tough cord or band of fibrous tissue by which

muscles are attached to bonetendinitis (tendonitis) inflammation of a tendonTENS (transcutaneous electrical nerve stimulation) a

type of therapy used to relieve pain that involvespassing electricity to nerve cells through electrodesplaced on the skin

TNF (tumor necrosis factor alpha) a cytokine (mes-senger protein) that plays a key role in the body’simmune response by promoting inflammation, con-trolling the production of other pro-inflammatorymolecules, and also helping the cells heal or repairthemselves. It attaches to a cell surface protein calledTNF receptor to exert its effect on the cell.

traditional Chinese medicine (TCM) An ancientChinese system of medicine, that includes meditation,herbal and nutritional therapy, restorative physicalexercises and massage, and acupuncture. (See alsoacupuncture; alternative healthcare and complemen-tary remedies)

urethritis an inflammatory condition of the urethra(the tube through which the urine travels from thebladder to the outside during urination)

ulcer a sore on the skin surface or on the inside liningof a body part, such as the mouth or stomach

ulcerative colitis an inflammatory disease of the innerlining of the gut that usually involves the colon orrectum. (See also inflammatory bowel disease)

Yersinia a group of bacteria comprising many differenttypes that may cause intestinal infection and diarrhea.Enteric infections with Yersinia, Salmonella, Shigella, orCampylobacter are the most common triggers for re-active arthritis

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Journal articles

Bakker C, Hidding A, van der Linden S, Doorslaer E van(1994) Cost effectiveness of group physical therapycompared to individualized therapy for ankylosingspondylitis. A randomized controlled trial. Journal ofRheumatology 21: 264–268.

Ball J (1971) Enthesopathy of rheumatoid and ankylos-ing spondylitis. Annals of Rheumatic Diseases 30:213–223.

Banares A, Hernandez-Garcia C, Fernandez-Gutierrez B,Jover JA (1998) Eye involvement in the spondy-loarthropathies. Rheumatic Disease Clinics of NorthAmerica 24: 771–784.

Barlow J, Cullen L (1996) Parenting and ankylosingspondylitis. Disability. Pregnancy ParenthoodInternational 15: 4–5.

Benedek TG, Rodnan GP (1982) A brief history of therheumatic diseases. Bulletin on the Rheumatic Diseases32: 59–68.

Boyer GS, Templin DW, Bowler A and colleagues (1997)A comparison of patients with spondyloarthropathyseen in specialty clinics with those identified in acommunitywide epidemiologic study. Has the classic

References and furtherreading

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case misled us? Archives of Internal Medicine 157:2111–2117.

Braun J, Brandt J, Listing J and colleagues (2002).Treatment of active ankylosing spondylitis withinfliximab: a randomized controlled multicentertrial. Lancet 359: 1187–1193.

Braun J, Bollow M, Reminger G and colleagues (1998)Prevalence of spondyloarthropathies in HLA-B27positive and negative blood donors. Arthritis andRheumatism 41: 58–67.

Braun J, Bollow M, Sieper J (1998) Radiologic diagnosisand pathology of the spondyloarthropathies.Rheumatic Disease Clinics of North America 24:697–735.

Braun J, Khan MA, Sieper J (2000) Entheses and enthe-sopathy: What is the target of the immune response.Annals of Rheumatic Diseases 59: 985–994.

Brus H, van der Laar M, Taal E, et al. (1997) Compliancein rheumatoid arthritis and the role of formal educa-tion. Seminars in Arthritis and Rheumatism 26:702–710.

Bulstrode SJ, Barefoot J, Harrison RA, Clarke AK (1987)The role of passive stretching in the treatment ofankylosing spondylitis. British Journal ofRheumatology 26: 40–42.

Calin A, Nakache J-P, Gueguen A, Zeidler H, MielantsH, Dougados M (1999) Defining disease activity inankylosing spondylitis: is a combination of variables(Bath Ankylosing Spondylitis Disease ActivityIndex) an appropriate instrument? Rheumatology 38:878–882.

Callahan LF, Pincus T (1995) Mortality in the rheumaticdiseases. Arthritis Care Research 2: 1327–1332.

Court-Brown WM, Doll R (1965) Mortality from cancerand other causes after radiotherapy for ankylosingspondylitis. British Medical Journal 59: 327–538.

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Dalyan M, Guner A, Tuncer S, Bilgic A, Arasil T (1999)Disability in ankylosing spondylitis. DisabilityRehabilitation 21: 74–79.

Dougados M, Revel M, Khan MA (1998) Spondyl-arthropathy treatment: Progress in medical treat-ment, physical therapy and rehabilitation. Baillière’sClinical Rheumatology 12: 717–736.

Dougados M, Linden S van der, Juhlin R et al. (1991)The European Spondyloarthropathy Study Grouppreliminary criteria for the classification of spondy-loarthropathies. Arthritis and Rheumatism 34:1218–1227.

Ebringer A, Wilson C (1996) The use of a low starch dietin the treatment of patients suffering from ankylos-ing spondylitis. Clinical Rheumatology 15 (Suppl 1):62–66.

Feldtkeller E, Bruckel J, Khan MA (2000) Contributionsof the ankylosing spondylitis patient advocacygroups to spondyloarthritis research. CurrentOpinion in Rheumatology 12: 239–247.

Feldtkeller E, Khan MA, van der Linden S, van derHeijde D, Braun J (submitted) Age at disease onsetand diagnosis delay in HLA-B27 negative vs. posi-tive ankylosing spondylitis. Annals of RheumaticDisease (submitted)

Finkelstein JA, Chapman JR, Mirza S (1999) Occult ver-tebral fractures in ankylosing spondylitis. SpinalCord 37: 444–447.

François RJ, Braun J, Khan MA (2001) Entheses andenthesitis: a histopathological review and relevanceto spondyloarthritides. Current Opinion in Rheuma-tology 13: 255–264.

Franke A and colleagues (2000) Long-term efficacy ofradon spa therapy in rheumatoid arthritis—a ran-domized, sham-controlled study and follow-up.Rheumatology 39: 894–902.

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References and further reading

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Gran JT, Skomsvoll JF (1997) The outcome of ankylos-ing spondylitis: a study of 100 patients. BritishJournal of Rheumatology 36: 766–771.

Granfors K, Marker-Herman E, De Keyser P, Khan MA,Veys EM, Yu DT (2002) The cutting edge ofspondyloarthropathy research in the millennium.Arthritis and Rheumatism 46: 606–613.

Gratacos J, Collado A, Pons F and colleagues (1999)Significant loss of bone mass in patients with early,active ankylosing spondylitis: a followup study.Arthritis and Rheumatism 42: 2319–2324.

Heikkila S, Viitanen JV, Kautiainen H, Kauppi M (2000)Sensitivity to change of mobility tests; effect ofshort term intensive physiotherapy and exercise onspinal, hip, and shoulder measurements in spondylo-arthropathy. Journal of Rheumatology 27: 1251–1256.

Herman M, Veys EM, Cuvelier C, De Vos M, BotelbergheL (1985) HLA-B27 related arthritis and bowelinflammation. Part 2: Ileocolonoscopy and bowelhistology in patients with HLA-B27 related arthritis.Journal of Rheumatology 12: 294–298.

Hidding A, van der Linden S, Gielen X and colleagues(1994) Continuation of group physical therapy isnecessary in ankylosing spondylitis: results of a ran-domized controlled trial. Arthritis Care Research 7:90–6.

Holman H, Loric K (1987) Patient education in therheumatic diseases: pros and cons. Bulletin on theRheumatic Diseases 37(5): 1–8.

Kahn M-F, Khan MA (1994) SAPHO syndrome.Ballière’s Clinical Rheumatology 8: 333–362.

Khan MA (1992) Spondyloarthropathies. RheumaticDisease Clinics of North America 18: 1–276.

Khan MA (1995) HLA-B27 and its subtypes in worldpopulations. Current Opinion in Rheumatology 7:263–269.

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Khan MA (1998) Slow-acting anti-rheumatic drugs insevere ankylosing spondylitis [Editorial]. Journal ofClinical Rheumatology 4: 109–111.

Khan MA (2000) Patient-doctor. Annals of InternalMedicine 133: 233–235.

Khan MA (2001) My self-portrait. Clinical Rheumatology20: 1–2.

Khan MA, Khan MK (1982) Diagnostic value of HLA-B27 testing in ankylosing spondylitis and Reiter s syndrome. Annals of Internal Medicine 96:70–76.

Khan MA, van der Linden SM (1990) A wider spectrumof spondyloarthropathies. Seminars on Arthritis andRheumatism 20: 107–113.

Khan MA, Khan MK, Kushner I (1981) Survival amongpatients with ankylosing spondylitis: a life-tableanalysis. Journal of Rheumatology 8: 86–90.

Kidd BL, Cawley MI (1988) Delay in diagnosis of spon-darthritis. British Journal of Rheumatology 27:230–232.

Koh TC (1982) Tai Chi and ankylosing spondylitis – Apersonal experience. American Journal of ChineseMedicine 10: 59–61

Kraag G, Stokes B, Groh J, Helewa A, Goldsmith C(1990) The effects of comprehensive home physio-therapy and supervision on patients with ankylosingspondylitis: a randomized controlled trial. Journal ofRheumatology 17: 228–233.

Laiho K, Tiitinen S, Kaarela K, Helin H, Isomaki H(1999) Secondary amyloidosis has decreased inpatients with inflammatory joint disease in Finland.Clinical Rheumatology 18: 122–123.

Lau CS, Burgos-Vargas R, Louthreno W, Mok MY,Wordsworth P, Zeng QY (1998) Features of spondy-loarthritis around the world. Rheumatic DiseaseClinics of North America 24: 753–770.

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Lloyd ME, Carr M, Mcelhatton P, Hall GM, Hughes RA(1999) The effects of methotrexate on pregnancy,fertility and lactation. Quarterly Journal of Medicine92: 551–563.

Lorig KR, Mazonson PD, Holman HR (1993) Evidencesuggesting that health education for self-manage-ment in patients with chronic arthritis has sustainedhealth benefits while reducing healthcare costs.Arthritis and Rheumatism 36: 439–446.

McGonagle D, Khan MA, Marzo-Ortega H, O’Connor P,Gibbon W, Emery P (1999) Enthesitis in spondy-loarthropathy. Current Opinion in Rheumatology 11:244–250.

Minden K, Kiessling U, Listing J, Niewerth M, Doring E,Meincke J, Schontube M, Zink A (2000) Prognosisof patients with juvenile chronic arthritis and juve-nile spondyloarthropathy. Journal of Rheumatology27: 2256–2263.

NIH (1998) NIH Consensus Development Panel onAcupuncture. Journal of the American MedicalAssociation 280: 1518–1524.

Ostensen M, Ostensen H (1998) Ankylosing spondylitis– the female aspect. Journal of Rheumatology 25:120–124.

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Pato E, Banares A, Jover JA and colleagues. (2000)Undiagnosed spondyloarthropathy in patients pre-senting with anterior uveitis. Journal of Rheumatology27: 2198–2202.

Prieur AM (1998) Spondyloarthropathies in childhood.Baillière’s Clinical Rheumatology 12(2): 287–307.

Reveille JD, Ball EJ, Khan MA (2001) HLA-B27 and genetic predisposing factors in spondylo-arthropathies. Current Opinion in Rheumatology 13:265–72.

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Roldan CA, Chavez J, Wiest PW, Qualls CR, CrawfordMH (1998) Aortic root disease and valve diseaseassociated with ankylosing spondylitis. Journal of theAmerican College of Cardiology 32: 1397–1404.

Rosenberg AM (2000) Juvenile onset spondylo-arthropathies. Current Opinion in Rheumatology 12:425–429.

Santos H, Brophy S, Calin A (1998) Exercise in ankylos-ing spondylitis: How much is optimum? Journal ofRheumatology 25: 215–60.

Sochart DH, Porter ML (1997) Long-term results of totalhip replacement in young patients who had anky-losing spondylitis. Eighteen to thirty-year resultswith survivorship analysis. Journal of Bone and JointSurgery (America) 79: 1181–1189.

Strobel ES, Fritschka E (1998) Renal diseases in anky-losing spondylitis: review of the literature illustratedby case reports. Clinical Rheumatology 17: 524–530.

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Tico N, Ramon S, Garcia-Ortun F, Ramirez L, Castello T,Garcia-Fernandez L, Lience E (1998) Traumaticspinal cord injury complicating ankylosing spondyli-tis. Spinal Cord 36: 349–352.

Uhrin Z, Kuzis S, Ward MM (2000) Exercise and changesin health status in patients with ankylosing spondyli-tis. Archives of Internal Medicine 160: 2969–2975.

van der Heijde D, Calin A, Dougados M, Khan MA, vander Linden S, Bellamy N (1999) Selection of instru-ments in the core set for DC-ART, SMARD, physi-cal therapy, and clinical record keeping inankylosing spondylitis. Progress report of the ASASWorking Group. Assessments in AnkylosingSpondylitis. Journal of Rheumatology 26: 951–954.

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van der Linden SM et al. (1984) The revised New Yorkcriteria for ankylosing spondylitis. Arthritis andRheumatism 27: 361–368.

van der Linden SM, Valkenberg HA, de Jongh B, Cats A(1984) The risk of developing ankylosing spondyli-tis in HLA-B27 positive individuals. A comparisonof relatives of spondylitis patients with the generalpopulation. Arthritis and rheumatism 27: 241–249.

van Royen BJ, De Gast A (1999) Lumbar osteotomy forcorrection of thoracolumbar kyphotic deformity inankylosing spondylitis. A structured review of threemethods of treatment. Annals of Rheumatic Diseases58: 399–406.

Ward MM (1999) Health related quality of life in anky-losing spondylitis. A survey of 175 patients. ArthritisCare Research 12: 247–255.

White M, Dorman SM (2001) Receiving social supporton line: implications for health education. HealthEducation Research 16: 693–707.

Yagan R, Khan MA (1983) Confusion of roentgeno-graphic differential diagnosis between ankylosinghyperostosis (Forestier’s disease) and ankylosingspondylitis. Clinical Rheumatology 2: 285–292.

Zeidler H, Mau W, Khan MA (1992) Undifferentiatedspondyloarthropathies. Rheumatic Disease Clinics ofNorth America 18: 187–202.

Books and monographs

Calin A, Taurog JD (eds) (1998) The spondylarthritides.Oxford University Press, Oxford.

Khan MA (1990) Ankylosing spondylitis and relatedspondyloarthropathies. In: Spine: State of the artreviews. Hanley & Belfus, Philadelphia, PA.

Khan MA (1996) Ankylosing spondylitis: Clinical fea-tures. In: Klippel JH, Dieppe PA (eds) Rheumatology,2nd edition. Mosby, London, p. 6.

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Khan MA (1996) Back and neck pain. In: Bone RC (ed)Current practice of medicine. Churchill Livingstone,Edinburgh, pp. 1–14.

Khan MA (1998) Spondyloarthropathies. In: Hunder G(Ed). Atlas of rheumatology. Current Science,Philadelphia, pp. 5.1–5.24.

Lopez-Larrea C (ed) (1997) HLA-B27 in the developmentof spondyloarthropathies. RG Landes Company,Austin, TX.

van der Linden S (1997) Ankylosing spondylitis. In:Kelly WN, Harris ED, Ruddy S, Sledge CB (eds)Textbook of rheumatology, vol 2, WB Saunders,Philadelphia, pp. 969–982.

WHO (1980) International Classification of Impairment,Disabilities, and Handicaps. World HealthOrganization, Geneva.

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Achilles tendonitis 22, 103reactive arthritis 131

Actonel 67Actron 40acupuncture 54–6addresses 145–9Advil 40aerobic exercises 81age of onset

AS 1, 20psoriatic arthritis 134

alcohol 37alendronate 67Aleve 40alternative therapy, see

nontraditional therapyAmerican Football 81American Heart Association

62American Psychiatric

Association 86amitriptyline 40amoxicillin 62ampicillin 62amyloidosis of the kidneys 70Anaprox 39, 40anesthesia 63–4ankylosing hyperostosis 99Ankylosing Spondylitis

Association of BritishColumbia (ASABC) 145

Ankylosing SpondylitisAssociation of Ireland(ASAI) 147

Ankylosing Spondylitis CaringSociety of R.O.C. 149

Ankylosing Spondylitis Groupof New South Wales 145

Ankylosing Spondylitis Groupof Queensland 145

Ankylosing Spondylitis Groupof Western Australia 145

Ankylosing SpondylitisInternational Federation(ASIF) 82, 143

annulus fibrosus 104Ansaid 39antibiotics 140antigen-presenting cells 122antinuclear antibodies 97anti-TNF therapy 4, 12,

47–8, 88side-effects, possible 48–9sponydloarthropathies

138–9aortic valve incompetence

108apical fibrosis 109apophyseal joints 104archery 80aromatherapy 57arthroplasty (joint replacement)

61, 79, 89prophylaxis 62

Arthrotec 39Associação Nacional da

Espondilite Anquilosante(ANEA) 147

Associazione ItalianaSpondiloartrite Anchilosante(A.I.Sp.A) 147

atlantoaxial joint, spontaneoussubluxation of the 69

Australia 145Austria 145autosomes 114Axid 41Ayurveda 57azathioprine 140Azulfidine 43

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back paincauses other than AS 98–9course of AS 19early diagnosis of AS 14–16

badminton 80balls, Swiss exercise 24bamboo spine 19, 105basketball 80–1baths

before exercise 24radon 50

Bechterew, Vladimir von 7Bechterewreumatikernas

Intresseorganisation (BERI)148

bee venom 57Belgium 145Bextra 39, 42bicycling, stationary 25bio-feedback 56biologic response modifiers

(biologicals) 48–9bisphosphonates 67body contact sports 81bony ankylosis of the spine

104, 105bowling 81boxing 81braces 77, 87breast-feeding 42, 43, 45, 79burning out of AS 4bursae 106bursitis 106Butazolidin 40

calcitonin 67calcium supplements 67Campylobacter infection 128Canada 145–6cancer of pelvis and spine 99car driving 81–2, 89case history, typical 71–3Cataflam 39

cauda equina syndrome 69–70causes of AS 2, 111, 113

see also HLA-B27CD4+ T cells 123CD8+ T cells 113, 122cefazolin 62Celebrex 39, 42celecoxib 39, 42, 43cervical spine 9cervicitis 131chest pain 105childbirth 79, 87children

course of AS 3, 21–2limb joints, involvement of 1spondyloarthropathies 135–7

undifferentiated 137–8chiropractice 52, 89Chlamydia infection 128,

129, 140choline magnesium trisalicylate

39chondroitin sulfate supplements

53chronic inflammatory bowel

disease (IBD) 2, 14, 108,112treatment 141

cimetidine 41circinate balanitis 132clindamycin 62Clinoril 39Clostridium infection 128colitis, ulcerative 2, 14, 108,

126enteropathic arthritis 135

complementary therapy seenonstandard therapy

computed tomography (CT)96

conjunctivitis 130, 135Conner, Bernard 7, 8contact sports 81Coordinadora Nacional de

Espondilitis anquilosante148

copper bracelets 57corsets 87

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corticosteroids 88effectiveness 45–6iritis 107–8spondyloarthropathies 138,

141psoriatic arthritis 139

cortisone 88course of AS 3–4, 13–14,

19–20, 132–3children 21–2gender differences 20–1older people 21

COX1 42COX2 42–3COX-2 specific NSAIDs

42–3C-reactive protein (CRP)

97case history 72

Croatia 146Crohn’s disease 2, 14, 108,

112, 126enteropathic arthritis 135treatment 141

cross-country skiing 80cycling

long-distance 81stationary 81, 139

cyclo-oxygenase (COX)42–3

cyclosporin 139cytokines 46cytotoxic T cells 113, 122Czech Republic 146

Daypro 39Denmark 146depression 85–6Deutsche Vereinigung Morbus

Bechterew (DVMB) 146developing countries

juvenile AS 21limb joints, involvement of 1spondyloarthropathies 128

diagnosis 1

back pain, other causes of98–9

early 14–17HLA-B27 testing 117–18laboratory findings 97–8New York criteria 98radiology 95–6reactive arthritis 132

diclofenac 39diclofenac sodium plus

misoprostol 39diets 37, 53, 87diffuse idiopathic skeletal

hyperostosis (DISH) 99diflunisal 39dimethyl sulfoxide (DMSO)

57disalcid 39disease-modifying

anti-rheumatic drugs(DMARDs) 43

DISH 99diving, avoidance of 25DMSO (dimethyl sulfoxide)

57Dolobid 39dowager’s hump 66downhill skiing 81driving 81–2, 89drug therapy 37–8,

87–8effectiveness 38

corticosteroids 45–6COX-2 specific NSAIDs

42–3methotrexate 44–5NSAIDs 38–43sulfasalazine 43–4

new treatments 49–50TNF-based therapy

46–9osteoporosis 67storage of medications 50see also named drugs

Drustvo za ankilozirajocispondilitis Slovenije (DASS)148

dynamic posture 77

thefacts 185

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early symptoms 13–14diagnostic pointers 14–17

earning capacity, AS’s impact on82–4

Elavil 40elderly people, course of AS

21elimination diets 53employment, AS’s impact on

82–4Enbrel 48enteropathic arthritis 137enthesitis 22, 103–5

early detection 96spondyloarthropathies

childhood 136, 139psoriatic arthritis 134reactive arthritis 131undifferentiated 137

enthesopathy, see enthesitisepidural anesthesia 63erythrocyte sedimentation rate

(ESR) 97case history 72reactive arthritis 132

erythromycin 62esomeprazole 41Estratab 67estrogen 67etanercept 48ethnicity, see race and ethnicityetodolac 39European Spondyloarthropathy

Study Group (ESSG) 126,127

Evista 67Excedrin 40exercise and physical therapy

23–5, 88–9heat, application of 25–6muscle-strengthening and

stretching exercises 28–36posture 77, 78–9rheumatologist’s role 92–3spinal extension and deep

breathing exercises 26–7spondyloarthropathies 139

swimming 25see also sports

eye inflammation 2, 4, 14,107–8course of AS 20

facet joints 104facts about AS 1–4falls, avoiding 25, 75family history 2, 10–12

see also geneticsfamily life 79–80family studies, HLA-B27

123–4famotidine 41Feldene 39fenoprofen 39fertility 79, 87fiber optic laryngoscopes 63fibromyalgia 16, 21fibrosis, apical 109fibrositis 16, 21Flanders 145flurbiprofen 39Food and Drug Administration

(FDA), US 51Forestier’s disease 99Fosamax 67

gadolinium 96genetic counseling 115–17genetics 2, 10–12, 111–12

psoriatic arthritis 134see also HLA-B27, genetics

Germany 146girdle joints 14, 105

see also hipglomerulonephritis 70glucosamine supplements

53gluteal tuberosity 10golfing 81Great Britain 146guided imagery 56

186 thefacts

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H2–blockers 41health-related quality of life

84–6heart block 108heart complications 62–3, 84,

89, 108heat, application of 25–6,

139heel pain 4, 22, 102–3

reactive arthritis 131–3helper T cells 123herbal therapies 54hiking 80hip 105

arthroplasty 61, 79, 89fracture 66

history, AS in 7HLA (human leucocyte

antigens) 112–13, 122–3HLA-B27 2, 111–13

genetics 10–12, 114–15counseling 115–17family studies 123–4

research 122–3association with AS 122family studies 123–4prevalence in world population

118, 119–21types 118, 120

role in disease predisposition113–14

spondyloarthropathies 125childhood 136, 137psoriatic arthritis 135reactive arthritis 126, 128,

129, 133testing in disease diagnosis

117–18hockey 81holistic medicine 56homeopathy 53Hrvatsko drustvo za

ankilozantni spondilitis146

human leucocyte antigens(HLA) 112–13, 122see also HLA-B27

Hungary 146hydrotherapy 24, 89hypnosis 56

ibuprofen 39, 40IgA kidney disease 70ilium 10imagery, guided 56Imuran 140Indocid 39Indocin 39indomethacin 39, 42infections as triggers

AS 111reactive arthritis 126, 128,

129inflammatory bowel disease

(IBD) 2, 14, 108, 112treatment 141

infliximab 48, 141inheritance, see genetics;

HLA-B27, geneticsinterdisciplinary co-operation

92–3international AS organization

143Internet 59, 86, 144–50Ireland 147iritis, acute 2, 4, 14, 107–8

course of AS 20psoriatic arthritis 130reactive arthritis 130

Italy 147

Japan 147Japan Ankylosing Spondylitis

Club 147jaw 106joint replacement (arthroplasty)

61prophylaxis 62

juvenile AS 21–2juvenile spondyloarthropathies

135–7

thefacts 187

Index

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keratinocytes 133keratoderma blennorrhagica

131ketoprofen 39, 40, 43ketorolac tromethamnine 39kidney dysfunction 70Klebsiella bacteria 111Klub bechterevik•u 146knee 21, 22, 136Krebs, Hans Adolf 7kyphosis 62

laboratory findings 97–8case history 72

Landsforeningen af MorbusBechterew Patienter 146

lansoprazole 41laryngoscopes, fiber optic 63later manifestations of AS 70

neurological problems 69–70osteoporosis 65–7spinal fracture 67–9

leukemia 138limb joints, involvement of

14, 105–6literature, AS in 7living with AS 3

car driving 81–2depression 85–6employment and earning

capacity, AS’s impact on82–4

falls, avoiding 75family life 79–80posture 75–7

dynamic 77occupational 77–9

quality of life, health-related84–5

sports and recreationalactivities 80–1

local AS organizations143–49

Lodine 39

low-starch diets 53lumbar spine 9lung complications 63, 84,

108–9lung function testing 109

magnetic resonance imaging(MRI) 96undifferentiated

spondyloarthropathy 138magnets 57management of AS, see

treatmentManitoba Ankylosing

Spondylitis Association 145Marie, Pierre 7massage 52, 89meclofenamate 39Meclomen 39meditation 54MEDLINE Plus 59mefenamic acid 39meloxicam 39men, course of AS 20methotrexate

effectiveness 44–5spondyloarthropathies 138,

139, 140–1methyl sulfonyl methane

(MSM) 57Miacalcin 67Mobic 39Motrin 39, 40Mozgáskorlátozottak

Egyesületeinek OrszágosSzövetsége, Bechterew section148

muscle-strengthening andstretching exercises 28–36

myths about AS 3–4

nabumetone 39Nalfon 39

188 thefacts

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naloxone 55Naprelan 39Naprosyn 39naproxen 39, 40naproxen sodium 39national ankylosing spondylitis

organizations 143–9National Ankylosing

Spondylitis Society (NASS)146

National Center forComplementary andAlternative Medicine(NCCAM) 58

National Institute of MentalHealth 86

National Institutes of Health(NIH) 59

nature of AS 5–6developments in treatment

12family history 10–12history, AS in 7literature, AS in 7sacroiliac joint 10spine, structure of 9–10terminology 7–8

Nederlandse bond vanverenigingen van patiëntenmet reumatischeaandoeningen, CommissieMorbus Bechterew 147

Netherlands 147neurological problems

69–70New York criteria 98Nexium 41nimesulide 39nizatidine 41non-spinal (limb) joints,

involvement of 105–6non-steroidal anti-inflammatory

drugs (NSAIDs) 12, 87–8COX-2 specific 42–3effectiveness 38–43spondyloarthropathies 138

unresponsive to 140–1

nontraditional therapy 51–2,56–8diets 53finding out about 58–9homeopathy 53Internet 59traditional Chinese medicine

54–6Norsk Revmatikerforbund

(NRF)/Bekhterev 147North American Spondylitis

Consortium (NASC)149

Norway 147NSAIDs, see non-steroidal

anti-inflammatory drugsNuprin 40nutritional supplements 53

occupational posture 77–9oestrogen 67older people, course of AS

21omeprazole 41onycholysis 133Ontario Spondylitis Association

(OSA) 145–6organizations, AS

international 143national and local 143–9research 149–50

Orudis 39, 40Oruvail 39Osgood–Schlatter’s disease

138osteitis condensans ilii 99osteomalacia 99osteophytes 99osteoporosis 21, 65–8

back pain 99drug therapy 67

Österreichische VereinigungMorbus Bechterew (ÖVMB)145

oxaprozin 39

thefacts 189

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Pagets disease 99pamidronate 49paraplegia 89pelvis, cancer of the 99penicillin 62Pepcid 41peptides 112, 122–3personalized information cards

68–9phenylbutazone 12, 39, 40physical therapy, see exercise

and physical therapypink eye 130piroxicam 39placebo effect, nontraditional

therapy 52, 55plantar fasciitis 22, 103

reactive arthritis 134Ponstel 39Portugal 147posture 19, 75–7, 88

dynamic 77exercise and physical therapy

23, 24occupational 77–9

pregnancy 79, 87drug therapy 42, 43, 45, 79

Premarin 67Prempro 67Prevacid 41prevalence

AS 3HLA-B27 118, 120, 122reactive arthritis 129

Prilosec 41process of AS 101–2

enthesitis 103–5eye inflammation 107–8non-spinal (limb) joints,

involvement of 105–6other structures, involvement

of 106, 108–9progesterone 67prophylaxis 62prostatitis 131proton pump inhibitors 41

psoralen-photo-augmentedultraviolet A (PUVA)treatment 139

psoriatic arthritis 2, 126, 132,133–5childhood 137treatment 139–40

pubic junction 10pubis 10PUVA treatment 139

quadriplegia 68quality of life, health-related

84–5

race and ethnicityHLA-B27 117, 118, 119–21osteoporosis 66psoriatic arthritis 134reactive arthritis 133

radiation therapy 49–50, 89radiology 95–6radium chloride 50radon bath 50radon gas inhalation 49–50raloxifene 67Ramses II 7ranitidine 41reactive arthritis (Reiter’s

syndrome) 2, 126–28causes 111children 137diagnosis 133outcome 132–3prevalence 129symptoms 130–2treatment 140

recreational activities 80–1Reiter’s syndrome, see reactive

arthritisRelafen 39Remicade 48, 141remission 4, 14

190 thefacts

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research 122–3HLA-B27

association with AS 122family studies 123–4prevalence in world populations

118, 119–21types 118–21

organizations 149–50Reston, James 55rheumatoid arthritis 21rheumatoid factor 97rheumatologist’s role 91–2

interdisciplinary co-operation92–3

Rheumatrex 44, 138, 140risedronate 67rofecoxib 39, 42Rufen 40rugby 81

sacroiliac joint 10, 11X-rays 2

sacroiliitis 13diagnosis 16, 95–6enteropathic arthritis 135psoriatic arthritis 135reactive arthritis 133–4X-rays 2

sacrum 10S-Adenosylmethionine (SAM-e)

53Salazopyrin 43, 138Salmonella infection 128,

129, 137Salsalate 39SAPHO syndrome 99‘sausage digits’

psoriatic arthritis 134reactive arthritis 131

Scheuermann’s disease 99Schober test 16Schweizerische Vereinigung

Morbus Bechterew (SVMB)148

sclerosis 104SEA syndrome 136

selective estrogen receptormolecules (SERMs) 67

self-help groups 89see also organizations, AS

seronegative spondyloarthritis97

sex chromosomes 114Shigella infection 128, 137shoulder 105side-effects

anti-TNF therapy 49, 88corticosteroids 45methotrexate 44–5NSAIDs 40–2, 43sulfasalazine 43, 44

Singapore 148Singapore Ankylosing

Spondylitis Club (SASC)148

skiing 80, 81skin lesions 131sleep

drug therapy 40posture 75–7, 88

Slovenia 148slow-acting anti-rheumatic

drugs 43, 44, 140–1smoking 27, 37, 88snake venom 57snorkels 25soccer 81Society of Patients with

Ankylosing Spondylitis(Bechterew’s disease) 149

Spain 148spinal extension and deep

breathing exercises 26–7spine

bony ankylosis 104, 105cancer 99fracture 62, 65, 67–9, 84

caused bychiropractice/massage 89

mechanical deterioration98–9

radiation treatment 89structure 9–10surgery 62

thefacts 191

Index

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splints 87spondylitis

psoriatic arthritis 135reactive arthritis 132–3

Spondylitis Association ofAmerica (SAA) 149Straight talk on spondylitis

79–80, 82spondyloarthropathies 22,

125–6, 127childhood (juvenile)

135–7enteropathic arthritis 135psoriatic arthritis 133–5,

139–40reactive arthritis 126–33treatment 138–9

methotrexate 140–1of skin involvement in psoriatic

arthritis 139–40sulfasalazine 140–1

undifferentiated 137–9spondylodiscitis 69spondylosis 5spontaneous subluxation of the

atlantoaxial joint 69sports 23, 80–1stationary cycling 81, 139storage of medications 50Straight talk on spondylitis

79–80, 82Strümpell, Adolf 7sulfasalazine

effectiveness 43–4spondyloarthropathies 138,

139, 140–1sulindac 39support groups 89

see also organizations, ASsurgical treatment 62–3,

89anesthesia 63–4joint replacement

(arthroplasty) 61prophylaxis 62

spondyloarthropathies 139Sweden 148swimming 25, 80, 89

spondyloarthropathies 139Swiss therapeutic exercise balls

24Switzerland 148symptoms

AS 1, 13–14spondyloarthropathies

childhood 136–7enteropathic arthritis 135psoriatic arthritis 133–4reactive arthritis 130–21undifferentiated 137–8

syndesmophytes 104

Tagamet 41Tai Chi 54Taiwan 149temporo-mandibular joint 106tendinitis 106tendons 106tennis 80tenoxicam 39TENS 56terminology 7–8thalidomide 49thoracic spine 9tiaprofenic acid 39tibial tubercle 22, 136TNF 46–7

anti-TNF therapy 4, 12,47–8, 88side-effects, possible 48–9sponydloarthropathies 138–9

Tolectin 39tolmetin 39Toradol 39total hip arthroplasty (THA)

61, 79, 89tracheostomy 63traditional Chinese medicine

(TCM) 54–6transcutaneous electrical nerve

stimulation (TENS) 56treatment 2, 4

developments 12overview 87–9

192 thefacts

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spondyloarthropathies 138–9skin involvement in psoriatic

arthritis 11–2methotrexate 140–1sulfasalazine 140–1

see also drug therapy; exerciseand physical therapy;nontraditional therapy;surgical treatment

Trisilate 39tumor necrosis factor alpha, see

TNF

Ukraine 149ulcerative colitis 2, 14, 108,

126enteropathic arthritis 135

undifferentiatedspondyloarthropathy137–8

United Kingdom 146United States of America

149urethritis 130–1US Food and Drug

Administration (FDA) 51

uveitis, anterior 107valdecoxib 39, 42Valentini 7venom, bee and snake 57Vioxx 39, 42

vitamin supplements 53vitamin D 139

Vlaamse Vereniging voorBechterew-patiënten (VVB)145

volleyball 80–1Voltaren 39von Bechterew, Vladimir 7

walking 80Web sites 59, 86, 144–9Welty, Eudora 7women

course of AS 3, 20–1osteoporosis 65–6see also breast-feeding;

pregnancyWorld Health Organization

(WHO) 55World Wide Web 59, 86,

144–9

X-rays 2, 16, 95–6

Yersinia infection 128, 137

Zantac 41

thefacts 193

Index

AS-Ind(183-194) 5/29/02 5:57 PM Page 193

ankylosing spondylitis: thefacts spondylitis... · ankylosing spondylitis: the facts as-01(1-4)...

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