Brief Overview of the Spondyloarthropathies

Stacy P. Ardoin, MD, MSAdult and Pediatric Rheumatology

Wexner Medical Center at The Ohio State University

and Nationwide Children’s Hospital

Objectives

Identify the spondyloarthropathies (ankylosing spondylitis, reactive arthritis, psoriatic arthritis, enteropathic arthritis) and the clinical features they have in common.

Describe the relationship between the HLA-B27 antigen and the spondyloarthropathies.

Relate the general approach to treatment of the spondyloarthropathies

Objectives

Spondyloarthropathies

Family of chronic inflammatory diseases with common clinical features Ankylosing spondylitis (AS) Reactive arthritis Psoriatic arthritis Enteropathic arthritis Undifferentiated spondyloarthropathies

Common (1-2% population)

Spectrum of Spondyloarthropathies

ankylosingspondylitis

psoriaticarthritis

enteropathicarthritis

reactive arthritis

undifferentiated spondyloarthritis

Common: overall prevalence 1-2% of adult population

Common Clinical Features of Spondyloarthroapthies

Involvement of axial skeleton especially sacroiliac (SI joints)

Peripheral arthritis Lower limbs > upper limbs Often asymmetric, oligoarticular

Enthesitis Extra-articular features

Mucocutaneus Uveitis

Male predominance, familial clustering HLA-B27 association, absence of autoantibodies

How is SpA different from RA?

SpA RA Male > Female Female > Male

Asymmetric oligoarthritis, dominant in lower extremities

Symmetric polyarthritis; dominant in upper extremities

Usually RF negative 80% RF positive

Cervical, lumbosacral spine involvement

Cervical spine involvement

Inflammatory Back Pain

Insidious onset before age 45 yrs Persistence for at least 3 months Accentuation of back pain in

morning after waking or after prolonged rest

Back pain improves with exercise

Calin & Fries. NEJM 1975; 293: 835

SpondyloarthropathiesSpondyloarthropathies

InflammatoryPeripheral

Arthritis

Enthesitis

Definition: inflammation of tendon, ligament, joint capsule at site of attachment to bone

Clinical manifestations Tendonitis Fasciitis Dactylitis Spondylitis

Dactylitis

Also seen in sarcoidosis, sickle cell anemia

Spondyloarthropathies

Symptomatic anterior uveitis Likelihood of Iritis

Disease Percent•AS 20-30•Reiter’s 12-37•PsA w/ spondylitis 7-16•IBD 2-9•Undiff SA ND

Spondyloarthropathies

Urethritis or Cervicitis

Acute urethritis or cervicitis due to Chlamydia or Gonorrhea can trigger reactive arthritis.

In addition, some SpA patients may have noninfections circinate balanitis.

Schober Test

Ankylosing spondylitis (AS)

Epidemiology Males affected more than females Peak age at diagnosis: 20 and 40 years Affects up to 1% of adult population

Juvenile AS Onset at age < 16 years

AS: Clinical Diagnosis

Must have inflammatory arthritis of axial skeleton Most common symptom: Inflammatory low back

or buttock pain Can have peripheral arthritis, usually oligoarticular, and

enthesitis Extra-articular symptoms:

Acute, symptomatic iritis Ulcerations of gastrointestinal tract Rare: interstitial lung disease, aortic valve

insufficiency.

Little et al. Am J Med 1976; 60: 278-285.

Psoriatic Arthritis (PsA)

Epidemiology Affects males and females equally Peak age at diagnosis: 20 and 40 years Prevalence: 1-2 per 1000 adults Incidence: 6 per 100,000 adults/year

Juvenile PsA Onset at age < 16 years Considered a juvenile idopathic arthritis

subtype.

PsA: Clinical Diagnosis

Psoriasis Classic skin lesions Nail lesions (pits, onycholysis) Arthritis occurs before skin lesions in about 15% of

patientsInflammatory arthritis

Axial Peripheral - usually oligoarticular, can be polyarticular.

Often involves DIP joints unlike RA.EnthesitisExtra-articular: symptomatic uveitis

Psoriatic Arthritis

In this image, note the nail findings and swelling of DIP joints in a patient with PsA.

This image shows extensive and destructive joint involvement of finger joints in a patient with PsA.

Skin and Nail Changes in PsA

Classic silvery plaques over elbows and in umbilicus.

Nail pitting in PsA

Nail thickening, discoloration, onycholysis. This patient also has psoriatic plaques on hands and obvious DIP swelling.

Enteropathic Arthritis

Complication of inflammatory bowel disease (IBD) Arthritis can parallel IBD disease activity Arthritis can be axial, peripheral

Signs of Enteropathic Arthritis due to IBD

Pyoderma gangrenosum

Oral ulcers Erythema nodosum

Symptomatic uveitis

Reactive Arthritis

Definition – Aseptic peripheral arthritis occurring within one month of infection Infections: GI, urethral, cervical (other)

Extra-articular manifestations common Can be self limited or chronic

HLA-B27+ increases risk of chronicity

Reactive Arthritis

Radiographic Findings in the Spondyloarthropathies

Sacroiliitis: Can be unilateral or bilateral. See widening and irregularity of joint space, sclerosis, erosions.

Lumbar spine: bridging syndesmophytes, “bamboo spine”.New bone forms along the interosseous ligaments.

Enthesitis

Erosion

New bone

HLA-B27 and the Spondyloarthropathies

ConditionPercent of people with the

condition who are HLA-B27 positive

Ankylosing SpondylitisCaucasians: 90-95%African-Americans:50%

Reactive Arthritis 60-80%

Enteropathic Arthritis 60%

Psoriatic Arthritis 60%

Undifferentiated Spondyloarthropathy

20-25%

HLA-B27 in Different Populations

Population Prevalence

Haida Indians 50%

Eskimos 20%

Norwegians 16%

US Caucasians 8%

African-Americans 2%

Japanese <1%

HLA-B27: Is it important?

HLA-B27 not sufficient to cause disease but appears to increase risk

HLA-B27antigen may impact the way the immune system reacts to bacteria, especially in the gut.

Overzealous reactions to bacteria may trigger systemic inflammation.

Treatment Approach to Spondyloarthropathies

NSAID

sulfasalazine, methotrexate

(often ineffective for axial disease)

intraarticularsteroids

PT, OT

topical steroids

Anti-TNF agentsAnti-TNF agents are usually the most effective therapy for

moderate to severe spondyloarthropathies.

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Case 1

History: A 42 yr old man develops swelling, morning stiffness in his left ankle and low back stiffness for 1 hour every morning. He also has pain in heels and bottoms of feet. This has been going on for about 3 weeks. Seven weeks ago he had urethral discharge and pain on urination. This improved without treatment but persists.

Case 1 - continued

Other past medical history: None

Other symptoms: No fevers, weight loss. No nasal or oral ulcers. No rash. No GI symptoms. Dysuria improved.

Family history: He has an uncle with chronic back pain, worst in mornings.

Social history: He is a married accountant who’s had 3 sexual partners in the past year.

Case 1 - continued

Physical Exam:He has no fever and does not appear acutely ill.

His exam is normal except for the following findings:

Genitourinary exam: erythema at urethra, no discharge

Musculoskeletal exam – effusion of his left ankle, reduced range of motion of lumbar spine with tenderness over sacroiliac joints on palpation, tender and swollen Achilles tendons.

Case 1 - Labs

Complete blood count, liver and kidney tests are normal.

Marks of inflammation (sedimentation rate, c-reactive protein) are elevated

Arthrocentesis of left ankle is performed: Synovial fluid – WBC 24,000, culture and gram stain are

negative, no crystals.

Urethral swab studies show that he has Chlamydia

HLA-B27 positive

Case 1 - Management

He and partners are treated with antibiotics for Chlamydia urethritis. He is treated with a non-steroidal anti-inflammatory drug (NSAID) for 4 weeks and the arthritis and enthesitis resolve.

The fact that he is HLA-B27 positive increases the risk that his symptoms may recur and may become chronic with or without an infection.

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Case 2

History: 20 yr old man presents with incapacitating lower back pain and stiffness. These symptoms have been present and worsening for 6 months. He’s had no injury to his back. None of his other joints are bothering him. He denies other symptoms.

Case 2 - continued

Other medical history: None

Review of systems: He specifically denies fevers, rash, eye pain or redness, oral ulcers, abdominal pain, diarrhea, dysuria, urethral discharge.

Family history: His brother has Crohn’s disease.

Case 2 - continued

Physical Exam:

He appears uncomfortable.

His exam is normal except for: Tenderness at both sacroiliac joints on

palpation, decreased lumbar spine range of motion (reduced Schober’s test).

Case 2 - Labs

Blood count, liver and kidney tests are normal.

Markers of inflammation (sedimentation rate and c-reactive protein) are elevated

HLA-B27 positive

Case 2 – x-rays

He has sacroiliitis of bilateral SI joints

X-ray shows subchondral sclerosis and irregularities of the joint surface,

including erosions in the iliac side of the sacroiliac joints.

Case 2 – Management

He is started on a non-steroidal anti-inflammatory drug but this fails to control his symptoms adequately.

Because he has axial disease which usually does not respond well to methotrexate or sulfasalazine, he is started on etanercept, an anti-TNF drug with marked improvement in symptoms.

Thanks!