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Question Number: 38

CaseA 52-year-old woman has a history of progressive ulnar deviation and PIPenlargement. She also has lumbosacral spine and hip, knee, and shoulder

joint involvement. Two years ago, she required right knee

replacement. Eight years ago, she had nephrolithiasis with several recurrentepisodes. The kidney stones have not been analyzed. She notes that there

are dark stains on her undergarments. She has a long history ofrosacea. One week ago, she began treatment for rosacea with minocycline.

Physical examination shows bilateral bluish-gray sclerae, mild ulnar deviationof the fingers, bilateral 2nd, 3rd, and 4th PIP enlargement, and a modifiedSchober test of 10 cm to 13 cm. Examination of the face shows erythema,

papules, and dilated blood vessels.

Radiographs of the lumbar sacral spine reveal loss of disc height, disc

calcification, very thick syndesmophytes, and bamboo spine (see figurebelow).

Figure

QuestionWhich of the following is the most likely diagnosis?

A. Rheumatoid arthritis

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B. Minocycline-induced hyperpigmentation

C. Alkaptonuria

D. Hyperuricemia and gout

E. Ankylosing Spondylitis

Correct Answer

C

Answer Rationale

Alkaptonuria is an autosomal recessive inborn error of metabolism. The

enzyme homogentisic acid oxidase is deficient resulting in large volume

excretion of

homogentisic acid (HGA) in the urine and sweat. Urine that contains HGA

will turn dark as the acid is oxidized to melanin-like product. This may result

in dark stains on clothing and undergarments. HGA may also accumulate oncartilage, tendons, sclerae, skin, and intima of large vessels. Nephrolithiasis

is a manifestation of the disease. Alkaptonuria resembles ankylosing

spondylitis involving the spine and large joints, however, it differs in that the

sacroiliac joints (SI joints) are spared. Minocycline has been associated with

hyperpigmentation resembling alkaptonuria, however, urine has normal HGA

measurement.

Question Number: 39

Case

A 56-year-old man has a maculopapular rash over the lower extremities thatcomes and goes. He notes some edema when he has the rash. He hasfatigue and some joint pain but no paresthesias. He has been losing weight.Review of other systems is unremarkable. He takes no medications.

Examination shows a normotensive man with palpable purpura and noabnormal neurological or musculoskeletal features. Findings on the

remainder of the general examination are normal.

Laboratory data include:

CBC: Normochromic anemia

BUN: 32 mg/dlCreatinine: 1.9 mg/dlAST: 86 U/L

ALT: 94 U/LAlkaline phosphatase: 110 U/L

Total bilirubin: 0.8 mg/dl

ESR: 98 mm/hrANA: 1:40 speckled

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dsDNA: negativeANCA: negative

RF: 450Anti-CCP antibody: negativeTotal protein: 8.8 gm/dlUrinalysis: 1+ protein, 2+ blood, no casts

Findings on chest radiograph are normal.

QuestionBased on the information above, which of the following is the most likelycause of the patients elevated rheumatoid factor?

A. Rheumatoid arthritis

B. Sjgrens syndrome

C. Lupus

D. Hepatitis-associated cryoglobulinemia

E. Wegeners granulomatosis

Correct Answer

D

Answer Rationale

The patient is a middle-aged man with palpable purpura, which is highly

suggestive of vasculitis. There is little in the history to suggest infection as atrigger, and there are no symptoms referable to the upper respiratory tract,

which are present in about 80% of patients with Wegeners

granulomatosis. He is not taking any medications that might trigger a

hypersensitivity vasculitis. The examination is unrevealing other than

showing purpura, and the laboratory studies show renal insufficiency, an

active urinary sediment, hepatitis, a low titer ANA with negative anti-dsDNA

antibodies, and markedly elevated rheumatoid factor. The anti-CCP antibody

is negative, arguing against RA, especially in light of the absence of joint

inflammation.

Causes of very high levels of rheumatoid factor include rheumatoid arthritis,

Sjgrens syndrome, and cryoglobulinemia. Cryoglobulinemia can be caused

by autoimmune diseases, chronic infections, and lymphoproliferative

disorders. Type I cryoglobulins are comprised of monoclonal

immunoglobulins, and are associated with lymphoproliferative disorders.

Type II cryoglobulins contain a monoclonal and polyclonal component, and

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can be seen with autoimmune diseases and infectious disease like hepatitis

C. Hepatitis C infection, as well as other infectious diseases, produce mixed

cryoglobulins, composed entirely of polyclonal immunoglobulins.

Rheumatoid arthritis is not likely, given the absence of joint findings and

negative anti-CCP antibody. Sjgrens syndrome is in the differential

diagnosis, but there is little in the clinical history to suggest its presence.

Results of SSA and SSB antibodies are not given. Wegeners granulomatosis

is not likely given the absence of symptoms referable to the upper

respiratory tract and the negative ANCA, which are seen in the majority of

patients with Wegeners granulomatosis. Lupus is also a possibility but the

patient does not appear to fulfill sufficient criteria to support the diagnosis.

Question Number: 40

CaseA 30-year-old man has gradually increasing right knee pain for the past 21

days. He is a long-distance runner who averages 30 miles a week. He saysthe pain began after a speed work out. The pain is on the lateral aspect ofthe knee. It resolves with rest to some degree, but any running causes it toreturn and causes him to limp for a day or 2. He can also bring on pain by

standing on his right leg and flexing his knee. The knee does not lock.

Physical examination shows tenderness over the lateral knee and lateral tibialcondyle. There is a creaking over the tender area with knee flexion and

extension. The knee is stable and there is no effusion.

QuestionWhich of the following is the best course of action at this time?

A. Prescribe NSAID and avoid running

B. Inject the right knee with a depot-corticosteroid preparation

C. Inject the bursa with anserine

D. Apply an elastic bandage (ACE wrap) and have the patient use crutches for 4-6 weeks

Correct Answer

A

Answer Rationale

Iliotibial band syndrome is a common injury in long-distance runners that

usually develops with over-training and after vigorous running or hiking. The

treatment involves avoiding running and NSAID therapy. In severe cases,

physical therapy and local corticosteroid injection may be helpful.

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There is no role for intra-articular injection or avoiding weight bearing in this

condition.

An MRI would be done if one expects a meniscal tear or ligament

injury. These conditions are unlikely because the joint is stable and there is

no effusion, locking, or joint-line tenderness.

A bone scan would be indicated if one suspects a stress fracture.

Surgery is not indicated for this condition unless it is chronic and refractory

to all other therapies.

Question Number: 41

CaseA 6-year-old girl with polyarticular juvenile idiopathic arthritis has a negativerheumatoid factor and positive ANA.

Question

How often should she undergo slit-lamp examination to screen for chroniciritis?

A. Every three to four months for four years then every six months

B. Every six months for four years then every year

C. Every year for four years then every two years

D. When her eyes hurt

E. When she is moved to the front of the class because of difficulty seeing the blackboard

Correct Answer

A

Answer Rationale

Iritis in children is usually chronic and asymptomatic until extensive scarring

has occurred. Those at highest risk of developing iritis are children with

young onset oligoarticular disease and who are ANA positive. However,

polyarticular ANA positive disease also carries a significant risk of iritis,

although lower than the oligaorticular group, and should be screened at the

same intervals.

Question Number: 42

CaseA 38-year-old woman is undergoing evaluation of new-onset polyarthritis

involving the small joints of her hands. She also has had a recurrent

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urticarial eruption over her shins (see figure below) for the past 3-4days. Both symptoms developed over the past 6 months.

Review of systems is notable for the recent need to switch from contactlenses to traditional lenses because of persistent corneal irritation. Althoughher previous dental health was excellent, she has recently developed three

new caries. She takes no medications.

Physical examination shows mildly injected conjunctivae. Having just taken adrink of water, her oral mucosa is moist, but there is no salivary pool. There

are 3 recently restored dental caries, two at the gingival margin.There is nopalpable lymphadenopathy. Findings on pulmonary, cardiac, and abdominalexaminations are normal. Peripheral articular examination shows symmetricsynovitis affecting the wrists and MCP and PIP joints.


C3: 123 mg/dlC4: 32 mg/dl

Figure

Question

Which of the following additional findings is most likely in this patient?

A. Hilar adenopathy

B. Cryoglobulins

C. Anti-SSA/Ro antibody

D. Anti-thyroglobulin antibody

E. Anti-neutrophil cytoplasmic antibody

Correct Answer

C

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Answer Rationale

The patient has secondary Sjgren syndrome in the setting of what is most

likely rheumatoid arthritis, given the symmetrical involvement of the small

joints of her hands. Sjgren syndrome may have a variety of cutaneous

manifestations, including urticarial eruptions. Sjgren syndrome patients with

anti-SSA/Ro antibody are more likely to have such extraglandular

manifestations of their disease. Dental caries at the gingival margin, as seen

in this patient, are characteristic in patients with oral sicca symptoms,

autoimmune or otherwise.

Hilar adenopathy is seen in the Lfgren syndrome, a female-predominant

presentation of sarcoidosis that is also marked by inflammatory polyarthritis

and erythema nodosum. Sarcoidosis may also present with sicca symptoms.

The knees and ankles are the most commonly affected joints. However,

erythema nodosum is characterized by erythematous, tender but

nonpruritic - subcutaneous nodules over the shins. Cryoglobulins may be

seen in rheumatoid vasculitis, but the cutaneous eruption is typically more

acral in distribution and is not typically pruritic. Anti-thyroglobulin antibody is

associated with autoimmune thyroid disease, but not rheumatoid arthritis or

Sjgren syndrome. Anti-neutrophil cytoplasmic antibodies are associated

with necrotizing vasculitis, including Wegener granulomatosis and Churg-

Strauss syndrome, but not rheumatoid arthritis or Sjgren syndrome.

Question Number: 43

CaseA 38-year-old man has polyarticular arthritis. Despite his relative youth and

absence of any history of joint trauma, he has widespread arthritis affectinghis axial skeleton, shoulders, and hips. He reports no visible articular swellingor erythema or palpable heat. He has otherwise been in excellent health andhas no family history of early-onset arthritis, either inflammatory or

mechanical.

Physical examination shows blue-black pigmentation of both sclerae (seeFigure 1).

Radiographs of his spine (see Figure 2) reveal calcification of the

intervertebral discs and disc space narrowing.

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Figure

Figure 1 Figure 2

QuestionWhich of the following tests is most likely to confirm the diagnosis?

A. COMP haplotype analysis

B. FRZB 2-marker single-nucleotide polymorphism (SNP) haplotype analysis

C. Targeted type II collagen polymorphism analysis

D. Plasma homogentisic acid assay

Correct Answer

D

Answer Rationale

The patient has alkaptonuria, an inborn error of metabolism resulting in

inability to metabolize homogentisic acid. Homogentisic acid is deposited in

the soft tissues and cartilage causing ochronosis, darkening of the sclerae,

pinnae, and other soft tissues, as well as premature osteoarthritis. The urine

will also darken if exposed to air.

The COMP haplotype, FRZB 2-marker SNP haplotype, and type II collagen

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polymorphisms have all been associated with premature osteoarthritis, an

effect that varies with the gender and ethnicity of the patient and the specific

joint studied, but will not cause the pigmentary changes seen in ochronosis.

Question Number: 44

CaseA 55-year-old woman has noted a sensation of grit in her eyes for the past 2years. She has been using artificial tears 2 or 3 times a day, particularly

while watching TV or working at a computer. Within the past year, she hasalso noted dryness of her mouth. She keeps a bottle of water at her bedside

and must drink water frequently to aid in swallowing dry food. She has notnoted any recent increase in dental caries. She does not have a rash, jointpain, or fatigue. She does not have clinical evidence of systemic lupuserythematosus, rheumatoid arthritis, or scleroderma.


WBC: 5600/mm3

Hgb: 13.2 gm/dlPlatelet count: 253,000/ mm3ANA testing (multiplex assay): negative antinuclear antibodies, negative SS-A and SS-B antibodies

QuestionWhich of the following additional findings is an absolute requirement forestablishing the diagnosis of primary Sjgrens syndrome in this patient?

A. Schirmers I test with wetting of 7 mm in the right eye and 4 mm in the left eye.

B. Unstimulated salivary flow of 0.1 ml/min

C. Labial salivary gland focus score of 1

D. Absent salivary pool

E. Parotid sialography showing extensive sialectasis

Correct Answer

C

Answer Rationale

Although there have been several sets of diagnostic criteria for Sjgrens

syndrome over the past 40 years, the set that is currently accepted was

derived by an international consensus group in 2002. According to the 2002

International Consensus Criteria for Sjgrens Syndrome, the diagnosis of

primary Sjgrens syndrome requires sicca signs/symptoms in the presence

of either Sjgrens antibodies (SS-A and/or SS-B) or a positive labial gland

biopsy, defined by the presence of a focus score of 1 or more. With the

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exception of the absent salivary pool, the other tests (abnormal Schirmers,

unstimulated salivary flow, or parotid sialography) would provide objective

evidence of the ocular or oral component of the disease and at least one

would be required to establish a diagnosis of Sjgrens syndrome in concert

with the abnormal labial gland biopsy.

Question Number: 45

CaseA 34-year-old woman with a 1-year history of scleroderma is seen in yourpractice as a new patient after moving from another city. She has had

Raynauds phenomenon in her hands as well as skin tightening on the handsand face. She is currently taking lisinopril, 20 mg/day and ASA 325

mg/day. She is a nonsmoker and has no family history of autoimmunedisease. Recently, she has developed a persistent dry cough and she

becomes short of breath after walking one block.

Blood pressure is 135/70 mmHg. She has tightening of the skin on the faceand fingers with digital pitting but no cyanosis. Velcro crackles are present

at both lung bases and she has borderline tachycardia at 105 bpm. Lowerextremities are without edema. Findings on chest radiograph are

normal. Echocardiogram shows a normal ejection fraction and an estimatedPA pressure of 25 mmHg. She is referred to a pulmonary specialist who does

bronchoaveolar lavage, and the fluid from this procedure shows 4%neutrophils. Cultures and stains on the fluid are negative for infectiousagents.

Question

Which of the following medications should be initiated at this time?

A. Prednisone 60 mg/day

B. Cyclophosphamide 1-2 mg/kg po daily

C. Mycophenolate Mofetil 1000 mg twice daily

D. Rituximab 750 mg/m2 x 2 doses

E. Infliximab 3 mg/kg

Correct Answer

B

Answer Rationale

The use of cyclophosphamide in scleroderma lung disease is based on results

from the Scleroderma Lung Study that compared in a blinded fashion oral

cyclophosphamide to placebo. Data from this 1-year study showed a

statistically significant improvement in lung function parameters in patients

treated with cyclophosphamide. Improvements were also observed in

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dyspnea and in quality of life indices, and these improvements persisted in

the year following cessation of cyclophosphamide treatment. The patient in

this case has neutrophilia on BAL consistent with the presence of

alveolitis. Prednisone in high doses is relatively contraindicated in

scleroderma because of the risk of precipitating renal crisis. The use of

mycophenolate mofetil has been assessed only in limited retrospective

studies and data regarding utility of rituximab and infliximab are largely

limited to case reports. There have been some reports suggesting that TNF

blockade might induce a scleroderma-like syndrome.

Question Number: 47

Case

A 30-year-old man was given the diagnosis with of rheumatoid arthritis 2years ago after he was evaluated for intermittent episodes of migratory

painful polyarthritis involving the knees, ankles, and elbows. The initial

episode lasted two weeks and on recurrence, synovitis of both elbows andtenderness in the wrists and hands was noted. At that time, rheumatoidfactor was positive and anti-citrullinated peptide antibodies were

negative. Therapy was initiated with methotrexate up to 20 mg orally everyweek without improvement. Between episodes he has polyarthralgias. His

father died at age 36 years from a myocardial infarction. Findings onphysical examination are normal with the exception of small nodules on the

Achilles, olecranon, and tibial tubercle. His hands and foot films are normal.

QuestionWhich of the following tests is the most appropriate next step to establish the

diagnosis?A. Anti-citrullinated peptide antibody titer

B. Rapid plasma reagent test

C. Anti-endomysial tissue transglutaminase antibody titer

D. Fasting serum lipid profile

E. Anti-nuclear antibody titer

Correct Answer

D

Answer Rationale

Lipoprotein disorders result from defect in either the synthesis or catabolism

of lipoprotein. Familial hypercholesterolemia is an autosomal dominant

disorder caused by mutation in the gene encoding for low density lipoprotein

(LDL) receptor. Deficiency results in high concentration of LDL in connective

tissues and arterial beds causing xanthomas and atherosclerosis. In patients

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C4: LowRheumatoid factor: 298 U

CCP: < 10 U

Figure

QuestionWhich of the following is the most appropriate next step in management?

A. Add an anti-TNF agent

B. Increase methotrexate dose

C. Add anti- B cell therapy

D. Begin cyclophosphamide

E. Add aspirin

Correct Answer

D

Answer Rationale

This patient has a nailfold infarction and sensory symptoms. This should raise

concern about the possibility of the development of rheumatoid vasculitis

(RV). RV refers specifically to a protean, destructive inflammatory process

that is centered on the blood vessel wall itself and associated with substantial

morbidity. It may affect a wide range of blood vessel types, from medium

muscular arteries to somewhat smaller arterioles to postcapillary venules.

Within a given patient, clinical features of both medium- and small-vessel

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disease may be found. RV leads to necrosis, blood vessel occlusion, and

tissue ischemia in a manner that resembles other forms of systemic

vasculitis, particularly polyarteritis nodosa.

RV typically occurs in patients with long-standing, joint-destructive RA. In

one study, the mean duration between the diagnosis of RA and the onset of

vasculitic symptoms was 13.6 years. Presentations of RV within 5 years of

the RA diagnosis are very unusual. Patients with RV nearly always have

rheumatoid nodules and are typically strongly positive for rheumatoid

factor. RV usually develops at a time when the inflammatory arthritis is

"burned out," ie, when the erosive process that led to joint destruction has

become less active.

The areas of the body involved most commonly by RV are the skin, digits,

peripheral nerves, eyes, and heart. The most devastating form of RV is

characterized by a medium-sized vasculitis reminiscent of (and histologically

identical to) polyarteritis nodosa. Many of the clinical manifestations of RV

reflect this predilection for medium-sized vessels, even though purpura,

petechiae, and papules (manifestations of small-vessel involvement) may

also occur.

In one study of 32 patients with rheumatoid vasculitis, the presence of

cutaneous vasculitis, multifocal neuropathy,and depressed C4 levels were the

3 independent variables that best predicted mortality. This patient had all

three of these variables that suggests that the use of cyclophosphamide

would be appropriate for this patient.

Question Number: 49

CaseA 66-year-old man with a history of ongoing chronic alcohol abuse, comes to

the emergency department because of bilateral knee pain. Physicalexamination shows a thin man with stigmata of liver disease, poor hygiene,

several nonblanching ecchymoses on both upper and lower extremities (seepicture) and bilateral knee effusions. There are no skin abrasions or

ulcers. He is able to flex his knees to 95 degrees with discomfort; both

knees are warm to touch. Arthrocentesis of the right knee is performed andshows a hemorrhagic effusion. A nontraumatic tap of the left knee reveals ahemorrhagic effusion also. (Image 1)


CBC: WBC 5,000/mm3, hgb11.6 gm/dl; platelet count 95,000/mm3

PT: 10.7 seconds

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PTT: 36 secondsBUN: 24 mg/dl

Creatinine: 1.4 mg/dlAST: 96 U/LALT: 45 U/L

Figure

Question

Which of the following is the most likely cause of the hemarthrosis?A. Thrombocytopenia

B. Pigmented villonodular synovitis

C. Decreased ascorbic acid

D. Circulating anticoagulant

Correct Answer

C

Answer RationaleVascular fragility due to vitamin C deficiency may lead to hemarthrosis and

subperiosteal bleeding. Scurvy is a clinical syndrome seen with ascorbic acid

deficiency largely due to impaired collagen synthesis with disordered

connective tissue. Symptoms (occurring as early as three months after

deficient intake) include ecchymoses, bleeding gums, petechiae,

hyperkeratosis, Sjgren-like syndrome, arthralgias, and impaired wound

healing. Generalized systemic symptoms are weakness, malaise, joint

swelling, arthralgias, edema, coiled hair, depression, neuropathy, and

vasomotor instability. In the United States, ascorbic acid deficiency occurs

mostly in severely malnourished individuals, drug and alcohol abusers, orthose living in poverty. Although as many as 80 percent of hospitalized

alcoholics have mild thrombocytopenia, in most the platelet function remains

normal. Decreased production of coagulation factors occurs in hepatic failure

but this patients PT and PTT are normal, which also eliminates the possibility

of a circulating anticoagulant as the cause of the hemarthrosis. Pigmented

villonodular synovitis or diffuse tenosynovial giant cell tumor is a benign

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neoplasm that typically presents as an intra-articular tumor expanding along

the synovial surface into the soft tissue. Clinical presentation of PVNS

usually involves the knee with pain, swelling, and dysfunction. Bloody

effusions are common. These tumors present at a younger age with most

patients younger than 40, monoarticular, and without systemic

manifestations.

Question Number: 50

CaseA 36-year-old man has a 14- month history of intermittent pain and stiffness

in the hands and feet. One year ago, he was evaluated for the persistentsymptoms in his hands and feet. He had Achilles tendon pain. Occasionally

he would have lumbar spine pain; however, activity helped improve thesymptoms.

Physical examination one year ago showed swelling of the right 2nd and 4thtoes and the left 3rd finger. A modified Schober test was 10 cm to 15 cm.

Laboratory data one year ago include:

ESR: 25 mm/hr

Treatment with indomethacin provided incomplete resolution of thesymptoms; however, he was able to resume some of his daily activities.

Indomethacin was continued for two months and then discontinued.Methotrexate was then prescribed for treatment.

He now has had progressive low back pain and stiffness for the past 4months. He describes morning stiffness that lasts for 2 hours. Two monthsago, he had iritis that was treated with topical corticosteroids.

Physical examination today shows modified Schober test of 10 cm to 13

cm. The 2nd and 4th toes are swollen, but there is no tenderness.

Radiograph of the sacroiliac joint (SI joints) reveals erosive changes on theiliac sides.

Laboratory data now include:

ESR: 25 mm/hr

QuestionWhich of the following is the best treatment option at this time?

A. Leflunomide

B. Cyclosporine

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C. Methotrexate

D. Azathioprine

E. TNF alpha antagonist

Correct Answer

E

Answer Rationale

Ankylosing spondylitis affects mainly the axial skeleton. The majority of

patients will have sacroiliac joint involvement. If a patient has peripheral

joint arthritis then sulfasalazine, cyclosporine, methotrexate or Arava may be

effective. However, axial involvement is best treated with TNF alpha

antagonist. Concomitant use of methotrexate with TNF alpha antagonist has

not been found to be superior to TNF alpha antagonist monotherapy for

treatment of axial manifestations.

Question Number: 51

CaseA 19-year-old African-American man has joint pain and a positive ANA (1:80

dilution). Approximately 3 years ago, he started having intermittentepisodes of severe bilateral knee, ankle, and hip pain. During these episodeshe has difficulty getting around. They occur 2 to 3 times a year, last for 2 to

3 days, and resolve spontaneously. He has not had any associatedsymptoms, such as fever, rash, swollen joints, or cardiopulmonaryproblems. NSAIDs, oxycodone, and morphine did not provide any

benefit. He is a smoker, nondrinker, and denies any illicit drug use. Heplays golf but has no other physical activities. He has not traveled outside

the USA. His mother has systemic lupus erythematosus. Findings onphysical examination are normal.


Hgb: 11 gm/dl

Hct: 36%ENA: negative

Chemistry profile: normal

Urinalysis: normalPlain radiographs of chest and knees: normalMRI of the right knee: Infarct of the medial femoral condyle, small infarct ofthe anterior distal femoral metaphysis and multiple small areas of edema.

QuestionWhich of the following studies is most likely to establish the diagnosis?

A. Bone marrow biopsy

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B. Bone scan

C. Hemoglobin electrophoresis

D. Anti-native DNA antibodies

E. Antiphospholipid antibodies

Correct Answer

C

Answer Rationale

The clinical diagnosis of osteonecrosis is appropriately made in a

symptomatic patient when imaging findings are compatible with this disease

and other causes of pain and bony abnormalities are either unlikely or have

been excluded by appropriate testing as in this case. Although there are

many conditions that lead to avascular necrosis, the most likely cause in this

case is hemoglobinopathy. Clues to this are young age of onset, the painfulcrisis, his ethnicity, and lack of systemic symptoms. Hemoglobin

electrophoresis will help to determine the cause of the painful crisis

associated with bone infarct. Long-bone infarct occurs most commonly with

HgBSS (sickle cell disease) but also with HbSC, HbS alpha thal, and least

commonly in sickle cell trait.

Bone marrow biopsy would be indicated if there were clinical findings

suggestive of Gauchers disease. Gauchers disease is a hereditary

(autosomal recessive) disorder of glucocerebroside metabolism that results in

the accumulation of cerebroside-filled cells within the bone marrow. Thisprocess may lead to compression of the vasculature and subsequent

osteonecrosis (60% of cases). Patients with Gauchers disease usually have

hepatosplenomegaly, thrombocytopenia, and bleeding, which are not present

in the above case. Bone scan will help to confirm osteonecrosis and identify

other asymptomatic areas but would not provide the cause. Patients with

classic antiphospholipid antibody syndrome present with venous and/or

arterial thrombosis and not with isolated osteonecrosis. A native-DNA

antibody is not indicated as nothing in this patients history or examination

points to a systemic autoimmune process.

Question Number: 52

CaseA 58-year-old man has a 3-year history of progressive muscle weakness

involving his shoulder and pelvic girdles. He had some difficulty swallowingliquids. He has been treated for 3 months with prednisone 60 mg/day withno benefit.

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Physical examination shows 4/5 strength in shoulder abduction and hipflexion.

Previously, he has had CK levels that were 3 to 4 times the upper range ofnormal. Electrophysiologic studies revealed increased insertional activity,fibrillation, and positive waves. Motor units were of low amplitude and short

duration. A muscle biopsy showed some atrophic and triangular fibers witha few lymphocytes in and around muscle fibers. Centralized nuclei were seen

but no necrosis or regenerating cells were present. Vacuoles were seen insome fibers. Trichrome staining revealed rare ragged red change.

Histochemistry with ATPase staining revealed fiber type grouping, and Congored staining was positive in fibers with vacuoles.

QuestionIn addition to physical therapy for muscle strengthening, which of thefollowing management options is most appropriate at this time?

A. Taper the prednisone

B. Sart azathioprine after checking thiopurine methyltransferase (TPMT) activity and slowly taperprednisone

C. Start methotrexate and azathioprine after checking thiopurine methyltransferase (TPMT) activityand slowly taper prednisone

D. Add IVIg

E. Add methotrexate

Correct Answer

A

Answer Rationale

Muscle histology in patients with inclusion body myositis may look identical to

that from patients with polymyositis; that is, degenerating and regenerating

muscle fibers with lymphocytes infiltrating the muscle and inside muscle

fibers. Over time, these inflammatory changes tend to resolve. In addition,

Trichrome staining may show ragged red change within some fibers. This

change is indicative of mitochondrial abnormalities and would suggest a

mitochondrial myopathy. However, ragged red fibers are reported with aging

and a mitochondrial myopathy would not be associated with the

inflammatory changes seen in this patient. In addition, patients with

inclusion body myositis may have neuropathic abnormalities. On physicalexamination, these may manifest as distal or asymmetric muscle

weakness. In muscle tissue, these are indicated by finding triangular cells

and fiber type grouping. Amyloid, as demonstrated with Congo red staining,

is present in IBM muscle fibers and in some vacuoles.

Although it is appropriate to give a trial of immunosuppressive therapy to

patients with presumed inclusion body myositis, these agents should be

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discontinued when it is clear that the patient is not responding because

inclusion body myositis is refractory to these medications.

Question Number: 53

CaseA 46-year-old man has had hypertension and diabetes mellitus for 20 yearsand has now developed chronic kidney disease. Ten years ago, he had whatappears to have been his first attack of gout. Synovial fluid aspirated from

his right knee at the time of a second attack contained monosodium uratecrystals. Over the next few years, he had multiple attacks of gout and visible

tophi developed on his elbows.

Two years ago, when his serum creatinine level was 2.0 mg/dl, he wasprescribed allopurinol 100 mg. This treatment led to a drop in his serumurate level from 11.0 mg/dl to 9.0 mg/dl. One year ago, the dose ofallopurinol was increased to 300 mg and colchicine 0.5 mg every other day

was added.

The diabetes mellitus is managed with insulin, he is takinghydrochlorothiazide 12.5 mg/day and losartan 25 mg for blood pressure

control, and he takes ASA 81 mg daily for cardioprotection.

He has not had a gout flare in the past 9 months but the tophi remainunchanged. Currently blood pressure is 165/100 mmHg, creatinine level is

2.3 mg/dl, and serum urate is 6.9 mg/dl (normal urate level in men is listedas 3.6-8.5 mg/dl).

QuestionWhich of the following interventions is most appropriate at this time?

A. Discontinue colchicne

B. Switch losartan to lisinopril

C. Discontinue the ASA

D. Add probenecid 1 g BID

E. Increase allopurinol to 400 mg/day

Correct Answer

E

Answer Rationale

Although many laboratories list the upper limit of normal for serum urate

levels above 6.8 mg/dl, the true physiochemical upper limit of normal of

serum urate is 6.8 mg dl. Above this concentration, body fluids are

supersaturated with urate and crystals can form and deposit. When levels

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above 6.8 mg/dl are listed as normal, they are based on statistical norms for

a population and are misleading regarding the pathophysiology of the

gout. To eliminate attacks and shrink tophi, serum urate levels must be

maintained below 6.8 mg/dl and preferably below a target value of 6.0

mg/dl.

Renal dysfunction increases the effectiveness of a given dose of allopurinol

because it delays the excretion of its active metabolite from the

body. Accordingly, the dose of allopurinol that is effective is usually lower

than that required for patients with normal renal function. Regardless, the

appropriate management of hyperuricemia in gout patients is to use the

lowest dose of whatever urate-lowering agent is used to maintain the serum

urate below 6.0 mg/dl.

Cochicine-induced axonal neuromyopathy most commonly occurs when renal

dysfunction is present, but it is reversible not permanent.

Losartan is an ideal antihypertensive agent for gout patients because it has

modest uricosuric effects. Hypertension in diabetic patients should be

managed with both an ACE inhibitor (such as lisinopril) and an ARB (such as

losartan). Increasing the dose of hydrochlorothiazide would not improve

blood pressure control but would induce a diuresis and have a hyperuricemic

effect.

Probenecid is not an effective agent in patients with renal dysfunction.

Question Number: 54

Case

A 52-year-old woman has recently received the new diagnosis ofosteoarthritis in the knees, which was made by her primary carephysician. She is otherwise generally healthy. She was a very active runnerfor many years and participated in 5K races until about 2 years ago. Now

she just jogs recreationally because she is somewhat limited by pain in herknees. She does not recall that anyone in her family had arthritis, and she

wants to know if there is anything she can do to improve her prognosis.

She had a hysterectomy 3 years ago. Her current medications include anestrogen patch and calcium supplements. She does not have diabetesmellitus or hypertension. She has smoked about a pack of cigarettes daily

for many years.

On examination, blood pressure is 135/72 mmHg and heart rate is 72/min.

She weighs 145 pounds and her height is 5 feet 5 inches; her calculated BMIis 24.1. She has crepitance in both knees, but the joints have full range of

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motion. There are no effusions in the knees. She has normal quadricepsmass and strength bilaterally.

Radiograph of the knee is shown in figure below.

Figure

QuestionWhich of the following has most likely contributed to cartilage loss in this

patient?

A. BMI of 24.1

B. Jogging

C. Use of cigarettes

D. Use of the estrogen patch

Correct Answer

C

Answer Rationale

While weight can impact osteoarthritis of the knee, this woman has a normal

BMI, so loss of weight is not likely to have significant benefit. Several

studies have now discounted the role of jogging or distance running in

accelerating the progression of knee OA. Furthermore, strength of the

quadriceps muscles that can be developed by this kind of exercise appears to

be an important factor in protection of the knee joint. Recent data suggest

that smoking is a modifiable risk factor for cartilage loss, so quitting cigarette

use is something that she can do to improve her joint health. Estrogen

supplements may slow rather than accelerate cartilage loss, so discontinuing

the patch probably will not help her joints.

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Question Number: 55

CaseA 28-year-old woman with a history of systemic lupus erythematosus,manifesting in the past as pleurisy, arthralgia, malar rash, and prior secondtrimester fetal loss, now has a positive pregnancy test. Her last menstrual

period was eight weeks ago. She feels fatigued but has no othersymptoms. She takes hydroxychloroquine 400 mg once daily and a prenatal

multivitamin. On physical examination, blood pressure is 110/70 mmHg andpulse rate is 88 bpm. No rashes are present.


ANA: 1:1280; homogeneous pattern

Urinalysis: trace protein; no RBC or WBC casts or cells/hpfSerum creatinine: 0.9 mg/dl

Hgb: 11.3 mg/dl

WBC: 3400/mm3Platelet count: 122,000/mm3APTT: 56 sec

Anticardiolipin antibodies: IgG 58 GPL units; IgM 11 MPL units

QuestionWhich of the following is the most appropriate additional treatment for this

patient at this time?

A. Aspirin 81 mg per day

B. Aspirin 325 mg per day

C. Heparin 10,000 IU sq bid and prednisone 60 mg orally daily

D. Heparin 10,000 IU sq bid and aspirin 81 mg per day

E. Prednisone 60 mg orally daily

Correct Answer

D

Answer Rationale

This patient is in her first trimester of pregnancy and has a history of

antiphospholipid syndrome with moderate titer anticardiolipin IgG antibody

positivity and prior fetal loss. Her risk for miscarriage is increased. Studies

comparing heparin and aspirin versus aspirin alone show superiority of

combination therapy. Prednisone use poses greater risk for corticosteroid

complications without improvement in outcome.

Question Number: 56

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CaseA 72-year-old woman has had a 3-month history of an erythematous rash

over her face, forearms, and upper torso. Serologic testing revealed thepresence of ANA (1:640, speckled pattern) and hydroxychloroquine 400mg/day was prescribed. She has had no joint swelling or muscle weaknessbut has had some mild dysphagia for liquids. Physical examination shows

normal blood pressure; there is a confluent erythematous papular rash overthe upper torso and sun-exposed areas of her arms extending onto the

dorsal aspect of the hands with periungual erythema and mild scaleformation over the dorsal PIP and MCP joints. Erythema is also present over

the sun-exposed areas of the face and eyelids. Oral and ocular membranesare normal and lungs are clear without audible rub or crackles. Joints arenormal without limitation or synovitis. Strength in the upper and lowerextremity muscle groups is normal.


CBC: normalChemistry profile: normalUrinalysis: normal

CK: normalAldolase: normal

Barium swallow radiograph: no mass, abnormal peristalsis in upper

esophagusChest, abdominal and pelvic CT scans: normal

Question

Which of the following studies is most appropriate at this time?A. Biopsy of affected dermis

B. EMG-directed muscle biopsy of an extremity muscle

C. MRI of the shoulder and pelvic girdle muscles

D. Vaginal ultrasonography and Ca-125 antigen study

E. Serologic tests for anti-dsDNA and anti-SSA

Correct Answer

D

Answer Rationale

Erythematous rash occurring on the face, eyelids, neck, and upper torso

along with papular lesions on the dorsum of the hands with periungual

erythema are very characteristic of dermatomyositis. The presence of orbital

violaceous erythema (heliotrope rash) and erythematous papules over the

dorsum of the hands is sufficient to establish the diagnosis of

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dermatomyositis. Skin biopsy frequently reveals interface dermatitis similar

to that seen in lupus and, for patients with these classic cutaneous

manifestations, adds little information to guide management. For patients

with demonstrable muscle weakness or CK elevation in the context of these

characteristic skin lesions, muscle biopsy is also not required to establish the

diagnosis.

The myopathy of dermatomyositis may be quite indolent or not clinically

manifest (dermatomyositis cine myositis). Moreover, it is important to

recognize that significant motor weakness may occur in the absence of

demonstrable elevations in creatine kinase or aldolase levels. Dysphagia due

to inflammatory myopathy of the pharyngeal muscles and/or striated portion

of the upper esophagus may be the initial presenting symptom of motor

weakness in patients with dermatomyositis.

The development of dermatomyositis, particularly in elderly patients,

requires consideration of possible associated malignancy. As such, in patients

such as the one presented, appropriate studies to determine the presence of

underlying lung, gastrointestinal/abdominal, or pelvic malignancies should be

performed. The prevalence of ovarian cancers in female patients with

dermatomyositis is particularly increased relative to the expected occurrence

and ovarian tumors in the early stages may be difficult to detect on routine

CT scanning. Serum studies for ovarian tumor antigens (Ca125) and vaginal

ultrasonography should, therefore, be performed to look for ovarian tumors

in female patients with dermatomyositis who have not undergone

oopherectomy.

Initial treatment of dermatomyositis occurring in the context of malignancy is

directed toward treatment of the neoplasm. In the absence of malignancy (or

in patients with persistent dermatomyositis manifestations despite removal

or treatment of the tumor), the inflammatory myopathy usually responds

favorably to corticosteroids, methotrexate, or other immunosuppressive

medications such as azathioprine. The cutaneous manifestations of

dermatomyositis are not uncommonly refractory to treatment with

corticosteroids and conventional immunosuppressive therapies. Skin disease

as well as the myopathy (when refractory to corticosteroids, methotrexate,

and/or other immunosuppressives) often responds favorably to a course of

IVIG, usually at a dose of 2 gm/kg administered in divided doses over 2-4

days. Preparations stabilized with sugars such as sucrose carry a higher risk

for renal toxicity and are best administered slowly, not exceeding 0.5

gm/kg/day.

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Diabetes mellitus is a much less likely cause of underlying peripheral

neuropathy, given the low-normal fasting glucose level. There is no

association between folic acid deficiency and carpal tunnel syndrome.

Acromegaly, diagnosed by the detection of an elevated insulin-like growth

factor I level, is also unlikely to be the cause of the worsening peripheral

neuropathic symptoms given his slight build and fine features, as opposed to

the more coarse features and broad hands expected in an individual with the

onset of acromegaly after puberty.

Question Number: 58

CaseA 55-year-old woman with diabetic glomerulosclerosis started hemodialysis 3

years ago. One year ago, swelling of her feet, lower legs, and handsdeveloped. Within a few weeks, this swelling progressed to a woody

induration, leading to flexion contractures of her fingers. There was also

progression of the induration to the forearms, upper legs, and thighs.Currently the affected skin has a brawny induration with areas of a peaudorange appearance as well as deep furrowing and loss of skin appendages.Skin biopsy shows diffuse dermal fibroplasia with spindle cells extending intothe subcutaneous tissues. There are thick collagen bundles separated by

large clefts in the dermis. Increased mucin is present.

QuestionWhich of the following additional findings is most likely in this patient?

A. Monoclonal gammopathy

B. Peripheral eosinophilia

C. Normal sedimentation rate and C-reactive protein

D. Raynauds phenomenon

E. Myocardial fibrosis

Correct Answer

E

Answer Rationale

Nephrogenic systemic fibrosis (NSF), formerly known as nephrogenic

fibrosing dermopathy, has been observed exclusively in patients with renal

insufficiency, most but not all of whom have undergone dialysis for renal

failure. The disease was initially thought to be confined to the skin. However,

recent reports have documented involvement of the liver, myocardium,

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lungs, diaphragm, and skeletal muscle. Increased ESR and C-reactive protein

level are commonly present. Paraprotein is a feature of scleromyxedema but

is not seen in NSF. The disease has been linked recently to the use of

gadolinium-based contrast agents for magnetic resonance imaging.

Question Number: 59

CaseA 72-year-old woman has had right knee pain and stiffness for the past six

months when arising from sitting. The knee pain worsens when she climbsstairs. She has had no trauma. She has a history of gastroesophageal refluxdisease and hypertension for which she takes omeprazole and enalapril. Onphysical examination, her weight is 110 pounds. Blood pressure is 130/80

mmHg. Right knee crepitus, medial joint tenderness, and a fluid wave arepresent. Radiograph of the right knee shows mild joint space

narrowing. She is treated with three weekly intra-articular injections of hylanG-F 20.

Three days after her last injection, she has severe worsening of pain andswelling of the right knee. Arthrocentesis of the right knee is performed andreveals 55 cc of cloudy yellow fluid.

Synovial fluid analysis is performed and reveals the following results:

WBC: 74,000/ mm3, differential 85% neutrophils,15% lymphocytes

RBC: 100/mm3Crystal analysis: no crystals observedGram stain: negative

Culture: pending

QuestionWhich of the following is the most likely diagnosis?

A. Osteonecrosis

B. Pseudogout

C. Pseudoseptic reaction

D. Rheumatoid arthritis

E. Septic joint

Correct Answer

C

Answer Rationale

This patient has a pseudoseptic reaction to injection with a hyaluronate. This

type of reaction may occur in 11% of injections with hyaluronate and is most

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commonly noted with hylan G-F 20. This reaction usually occurs in the first

week after the injection. Nucleated cell counts may exceed 100,000/mm3

and are predominantly neutrophils. Iatrogenic septic joint after intra-

articular injection has been estimated to occur once in 2,000 to once in

15,000 injections. Some cases of crystal-proven pseudogout have been

reported to occur after injection of hylan G-F 20. However, this patients

synovial fluid did not contain any crystals. Osteonecrosis is not a

complication of hyaluronate injection and is not associated with highly

inflammatory synovial fluid. This patient does not meet criteria for a

diagnosis of RA.

Question Number: 60

CaseA 14-year-old girl has a 6-week history of unexplained low-grade fever,fatigue, and joint pain. Family history reveals that her 4-year-old brother

has a history of perirectal abcesses, two hospitalizations for staphylococcalpneumonia, and a chronic cervical lymphadenitis for which culture grewSerratia species.

Question

Which of the following is the most likely diagnosis in the sister?

A. Inflammatory bowel disease

B. Common variable immune deficiency

C. Endocardial elastosis

D. Systemic lupus erythematosus

E. Chronic EBV syndrome

Correct Answer

D

Answer Rationale

The x-linked form of CGD usually presents with perirectal abcess and

difficulties in clearing staph infection and infection caused by other catylase-

positive organisms. Having a brother with these symptoms should raise the

index of suspicion of SLE. More commonly, this is discoid lupus, but it canalso be systemic. In the absence of that diagnosis, IBD and CVID could also

cause similar symptoms and appropriate evaluation should be undertaken.

Inflammatory bowel disease may present with fever, fatigue, and arthralgia,

but the family history points more toward SLE. Common variable immune

deficiency (CVID) may also have similar features, but the age of onset and

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types of infections in the brother are not those usually found with CVID. The

carriers of CGD are also at higher risk for endocardial fibroelastosis, but the

symptoms would more likely be shortness of breath and cardiac failure. The

patient had no adenopathy or rash suggestive of EVB, and duration is too

long for this to be likely.

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