RHEUMATOLOGY MCQS- 2008 1-37

Question Number: 1

CaseA 70-year-old man with chronic obstructive pulmonary disease has a T8 vertebral compression deformity noted on a lateral chest radiograph taken for evaluation of a chronic cough. The deformity was not present on radiographs taken 1 year ago. The patient does not have back pain and does not recall any injury to his back. He has not been taking corticosteroids.

Laboratory data include:

Serum testosterone: 205 ng/ml DEXA bone densitometry: lumbar spine T-score = -1.8 Femoral neck T-score = -1.9

QuestionWhich of the following is the most appropriate therapeutic intervention at this time?

A. Increase calcium intake to 1200 mg/day with vitamin D 800 IU/d

B. Oral alendronate 70 mg once weekly in addition to supplemental calcium and vitamin D

C. Transdermal testosterone 5 mg at night in addition to supplemental calcium and vitamin D

D. Teriparatide 20 mcg SC daily in addition to supplemental calcium and vitamin D

E. Intranasal calcitonin, 200 units once daily

Correct AnswerB

Answer RationaleThe presence of a vertebral compression fracture is sufficient to establish a diagnosis of osteoporosis, even though the bone mineral density T-scores are in the osteopenic range. If the bone mineral density in a man is evaluated using reference data from a healthy female population, then osteoporosis will be significantly underdiagnosed. Given the presence of one vertebral fracture, this patient is at high risk of additional fractures and needs to receive drug therapy for his osteoporosis. The most effective therapies would be a bisphosphonate (e.g. alendronate) or teriparatide. Given the expense and inconvenience of teriparatide, alendronate is the most appropriate first therapy for this patient. Intranasal calcitonin has not been shown to reduce fracture risk in men with osteoporosis. This patient has a low normal testosterone level, most likely a consequence of an age-related decline in his gonadal function. The benefits of testosterone therapy for the prevention of osteoporotic fractures in hypogonadal men have not been established. There is also uncertainty as to whether testosterone replacement improves bone mineral density in older men with borderline low testosterone levels. Since

androgen therapy has possible undesirable effects on other organ systems, particularly the prostate gland, testosterone therapy would not be the first choice of therapy for this man.

Question Number: 2

CaseAn over-weight 14-year-old boy had been active at a soccer tournament yesterday and complained of right knee pain when he returned home. Today the pain is still present and worsens with weight bearing. He has been previously diagnosed with mild hypothyroidism, and sometimes he forgets to take his replacement therapy.

Physical examination shows no abnormalities of the knee, but internal rotation at the hips is limited.

Radiograph of the hips is shown in figure below.

Figure

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

A. Nonsteroidal anti-inflammatory drug

B. Antibiotics after aspiration for presumed septic hip

C. Non-weight bearing and orthopedic evaluation

D. Use of crutches for two weeks and weight bearing as tolerated

E. Physical therapy program to increase the range of movement of his hips

Correct AnswerC

Answer RationaleChildren often complain of knee pain when the hip is the source of the problem. SCFE is more common in obese adolescent boys and also in children with hypothyroidism. It is a surgical emergency; the child should be made non-weight bearing immediately, additional radiographs (including frog-leg lateral view) should be obtained, and urgent orthopedic consultation should be ordered. Treatment is surgical pinning. There is increased risk of slip on the other side, and some children develop aseptic necrosis or chondrolysis as a consequence of this condition.

Question Number: 3

CaseA 26-year-old man has had gradual onset of fixed proximal muscle weakness over the past 3 to 4 years. He had a healthy childhood but began experiencing exercise intolerance during his teen years. He often found that when he exercised, he would experience dyspnea and muscle pain shortly after beginning the activity. These symptoms would force him to stop what he was doing. However, after resting for 2 or 3 minutes, he could resume the activity without discomfort. On one occasion, he experienced severe cramping and muscle tenderness after going fishing and carrying his catch in a bucket of water back to car, a distance of about a half mile. The cramps lasted more than an hour and the muscle tenderness resolved in 48 hours.

On examination, he has 4/5 strength in proximal muscles of the upper and lower extremities. Findings on examination of the joints and the remainder of the neurologic examination are normal.

Laboratory studies reveal CK of 900 U/L. EMG shows fibrillation potentials and early recruitment of short-duration, low-amplitude motor unit action potentials. A decremental response was observed to repetitive 20-Hz nerve stimulation.

QuestionWhich of the following is the most likely finding on muscle biopsy?

A. Perivascular infiltration of lymphocytes with perifasicular atrophy and capillary plugging

B. Occasional degenerating and regenerating fiber with PAS positive subsarcolemmal vacuoles

C. Many small triangular fibers and fiber type grouping

D. Some type II fiber atrophy and increased oil red O staining in type I fibers

E. Many fibers with central nuclei, occasional hypertrophic cells, and ragged red changes on Gamori Trichrome staining

Correct AnswerB

Answer Rationale

McArdle’s disease is the most common glycogen storage disease. It may present in childhood with exercise intolerance; in teen years with episodes of acute rhabdomyolysis associated with severe cramps, tenderness, and weakness that often occur after heavy exertion; or in adulthood with the gradual onset of fixed proximal muscle weakness. The fixed weakness is felt to be the result of recurrent rhabdomyolysis and an inability, after a time, to regenerate normal muscle. Many patients report a “second wind” phenomenon. They start to exercise but must stop, only then to be able to resume the activity after a brief rest. This is presumably the result of increased blood flow and delivery of nutrients that occur in response to the initial exercise. Most of these patients have an elevated CK level even when asymptomatic and have myopathic changes on EMG. The finding of PAS positive vacuoles confirms the diagnosis of a glycogen storage disease.

Perivascular infiltration of lymphocytes with perifasicular atrophy and capillary plugging are classically seen in dermatomyositis.

Many small triangular fibers and fiber type grouping would be seen in a neuropathic condition and imply denervation and reinervation.

Some type II fiber atrophy and increase oil red O staining in type I fibers occurs with lipid storage diseases like carnitine deficiency of some mitochondrial myopathies.

Many fibers with central nuclei, occasional hypertrophic cell, and ragged red change on Gamori Trichrome staining are indicative of a mitochondrial myopathy.

Question Number: 3

CaseA 26-year-old man has had gradual onset of fixed proximal muscle weakness over the past 3 to 4 years. He had a healthy childhood but began experiencing exercise intolerance during his teen years. He often found that when he exercised, he would experience dyspnea and muscle pain shortly after beginning the activity. These symptoms would force him to stop what he was doing. However, after resting for 2 or 3 minutes, he could resume the activity without discomfort. On one occasion, he experienced severe cramping and muscle tenderness after going fishing and carrying his catch in a bucket of water back to car, a distance of about a half mile. The cramps lasted more than an hour and the muscle tenderness resolved in 48 hours.

On examination, he has 4/5 strength in proximal muscles of the upper and lower extremities. Findings on examination of the joints and the remainder of the neurologic examination are normal.

Laboratory studies reveal CK of 900 U/L. EMG shows fibrillation potentials and early recruitment of short-duration, low-amplitude motor unit action potentials. A decremental response was observed to repetitive 20-Hz nerve stimulation.

QuestionWhich of the following is the most likely finding on muscle biopsy?

A. Perivascular infiltration of lymphocytes with perifasicular atrophy and capillary plugging

B. Occasional degenerating and regenerating fiber with PAS positive subsarcolemmal vacuoles

C. Many small triangular fibers and fiber type grouping

D. Some type II fiber atrophy and increased oil red O staining in type I fibers

E. Many fibers with central nuclei, occasional hypertrophic cells, and ragged red changes on Gamori Trichrome staining

Correct AnswerB

Answer RationaleMcArdle’s disease is the most common glycogen storage disease. It may present in childhood with exercise intolerance; in teen years with episodes of acute rhabdomyolysis associated with severe cramps, tenderness, and weakness that often occur after heavy exertion; or in adulthood with the gradual onset of fixed proximal muscle weakness. The fixed weakness is felt to be the result of recurrent rhabdomyolysis and an inability, after a time, to regenerate normal muscle. Many patients report a “second wind” phenomenon. They start to exercise but must stop, only then to be able to resume the activity after a brief rest. This is presumably the result of increased blood flow and delivery of nutrients that occur in response to the initial exercise. Most of these patients have an elevated CK level even when asymptomatic and have myopathic changes on EMG. The finding of PAS positive vacuoles confirms the diagnosis of a glycogen storage disease.

Perivascular infiltration of lymphocytes with perifasicular atrophy and capillary plugging are classically seen in dermatomyositis.

Many small triangular fibers and fiber type grouping would be seen in a neuropathic condition and imply denervation and reinervation.

Some type II fiber atrophy and increase oil red O staining in type I fibers occurs with lipid storage diseases like carnitine deficiency of some mitochondrial myopathies.

Many fibers with central nuclei, occasional hypertrophic cell, and ragged red

change on Gamori Trichrome staining are indicative of a mitochondrial myopathy.

Question Number: 5

CaseA 55-year-old woman has had an aching pain in her lower back for the past 2 years.


Serum calcium: 8.2 mg/dlSerum phosphorus: 2.3 mg/dlAlkaline phosphatase: 175 U (normal 33-115 Units)25-hydroxyvitamin D: 8 ng/ml (normal 20-100 ng/ml)1,25 hydroxyvitamin D: 40 pg/ml (normal 25-45 pg/ml)Parathormone: 108 pg/ml (normal 10-65 pg/ml)

QuestionWhich of the following radiographic abnormalities is most likely in this patient?

A. Increased bone mineral density

B. Subperiosteal resorption

C. Looser’s zones

D. Erlenmeyer flask deformity of the distal femora

E. Mixed areas of lucency and sclerosis in the long bones

Correct AnswerC

Answer RationaleThis patient has osteomalacia as a result of vitamin D deficiency. Characteristically, both the serum calcium and the serum phosphorus levels are decreased, while the serum alkaline phosphatase level is elevated. In vitamin D deficiency, parathormone levels rise, causing an increase in the metabolism of 25-hydroxyvitamin D to 1,25-hydroxyvitamin D. This in turn results in a greater deficiency of vitamin D but also a normal or elevated level of 1,25-hydroxyvitamin D.

The characteristic radiographic feature of osteomalacia is Looser’s zones or pseudofractures that appear as linear areas of rarefaction perpendicular to the cortical surface of the bone. Increased bone mineral density is a feature of osteopetrosis. Subperiosteal resorption occurs in primary hyperparathyroidism. The Erlenmeyer flask deformity is a feature of Gaucher’s disease. Mixed areas of lucency and sclerosis in the long bones are a feature of Paget’s disease.

Question Number: 6

CaseA 21-year-old white woman has sudden onset of swelling and pain in her right knee associated with warmth and erythema. Synovial fluid analysis of fluid obtained from the knee reveals 21,000 WBC/mm3, and needle-shaped negatively birefringent crystals are found under polarized light microscopy.

Family history reveals that the patient’s father and brother have gout.


Serum creatinine : 2.9 mg/dl Serum urate: 10.5 mg/dl24-Hour urinary uric acid: 350 mg

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

A. She is homozygous for phosphoribosylpyrophosphate (PRPP) synthase overactivity

B. She is heterozygous for hypoxanthine-guanine phosphoribosyltransferase (HPRT) deficiency

C. She homozygous for adenine phosphoribosyltransferase (APRT) deficiency

D. She has a mutation in the uromodulin (UMOD) gene

E. She is homozygous for purine nucleoside phosphoylase (PNP) deficiency

Correct AnswerD

Answer RationaleBoth PRPP synthase overactivity and partial HPRT deficiency cause hyperuicemia and gout, but both have X-linked inheritance and, therefore, do not occur in women.

A deficiency of APRT causes affected individuals to have kidney stones composed of 2,8 dihydroxyadenine.

Mutations in UMOD gene are responsible for familial juvenile hyperuricemic nephropathy (FJHN). FJNG is inherited as an autosomal dominant trait with a high degree of penetrance and is usually associated with gout. Renal disease usually develops in the second decade of life and progresses to end-stage renal disease by mid-life.

PNP deficient individuals have selective T-cell immunodeficiency.

Question Number: 7

CaseA 52-year-old woman with seropositive erosive rheumatoid arthritis has increasing joint pain, swelling, and stiffness and has been under stress at work. She has been taking methotrexate for several months. On physical examination, she is anxious and has synovitis. ESR is 74 mm/hr. She is reluctant to take an anti-TNF blocker and agrees to start leflunomide in addition to continuing methotrexate.

After several weeks, the joint pain and stiffness improve and follow-up examination shows minimal synovitis; her mood is better. She returns for evaluation two months later and findings on examination are unchanged except she has lost 4 pounds. CBC and chemistries are normal and ESR is 30 mm/hr.

At her next visit 2 months later, she has lost an additional 6 pounds and feels well. Examination shows no synovitis or adenopathy.


CBC: normalESR: 26 mm/hrTSH: 0.9 µU/mlLFT: normalBUN: 23 mg/dlCreatinine: 1.0 mg/dlAlbumin: 4.0 gm/dl

QuestionWhich of the following is the most likely cause of the patient’s weight loss?

A. Active rheumatoid arthritis

B. Methotrexate associated lymphoma

C. Hyperthyroidism

D. Anxiety

E. Idiosyncratic effect of leflunomide

Correct AnswerE

Answer RationaleRheumatoid arthritis is a systemic inflammatory disease that affects the joints and extra-articular systems. Constitutional symptoms such as weight loss are not uncommon with uncontrolled disease. Patients with rheumatoid arthritis are at increased risk for lymphoma, and methotrexate is known to cause reversible lymphomas, but there is no indication that this patient has lymphoma. She does not appear to be clinically hyperthyroid, and her anxiety improved.

Leflunomide is a disease-modifying anti-rheumatic drug that works by inhibiting de novo pyrimidine biosynthesis. Actively dividing lymphocytes are inhibited by the drug’s effect on the enzyme dihydroorotate dehydrogenase, which is directly involved in pyrimidine biosynthesis. Weight loss caused by leflunomide is under recognized and may reflect the drug’s effect on oxidative phosphorylation within the mitochondria, rather than a secondary effect resulting from diarrhea or anorexia. Cessation of leflunomide may result in weight gain. The weight loss averaged between 19 and 53 pounds and occurred within weeks to months of beginning leflunomide. Recognition of this side effect is important since unnecessary additional testing may be undertaken to investigate weight loss.

Question Number: 8

CaseA 49-year-old man has progressive swelling of the left foot. He had noted tingling and burning in the foot at night for the past year. The foot then became swollen five months ago. One month ago, he began to note discomfort in the foot with ambulation. He does not have pain in any other joints. He has a 10-year history of diabetes mellitus type 2 and hypertension. The patient’s current medications are losartan, hydrochlorothiazide, metformin, glyburide, and simvastatin. He drinks one or two 12-ounce cans of beer on weekends. He is afebrile.

Physical examination is notable for pain, swelling, and erythema of the dorsum of the left foot and ankle. The midfoot and ankle are tender. There is restricted range of motion of the ankle with pain. Deep tendon reflexes are absent at both ankles. There is diminished vibration sensation in both feet and decreased proprioception of the toes of both feet. No ulcers or rash are present. Hemoglobin level is 10.4 gm/dl.

A radiograph of the left foot is shown below:

Figure

QuestionWhich of the following is the most likely diagnosis?

A. Calcium pyrophosphate dehydrate deposition disease

B. Chronic gout

C. Neuropathic osteoarthropathy

D. Osteomyelitis

E. Reflex sympathetic dystrophy syndrome

Correct AnswerC

Answer RationaleThis patient has a long-standing history of diabetes mellitus type 2 and months of foot swelling. The radiograph shows hindfoot and midfoot joint and osseous destruction, osteopenia, and reactive bone formation. No chondrocalcinosis is present making CPPD arthropathy unlikely. The radiographic changes are out of proportion to the pain the patient describes. This is unusual for reflex sympathetic dystrophy in which causalgic pain is usually prominent and allodynia typically present. Osteopenia and marked osseous destruction are not typical of osteoarthritis. This radiograph does not show typical features of gout including “punched out,” rounded defects marginally and erosions with overhanging edges. Osteomyelitis may be present in diabetic patients with foot ulcers, but this patient has no preexisting foot ulcer. The patient also

has no paronychia, cellulitic skin features nor constitutional symptoms including fever to suspect infection.

Question Number: 9

CaseAn 8-year-old boy has had hectic fevers for the past 5 days, a diffuse morbilliform rash on his trunk, swollen hands and feet, erythematous oral mucosa with no exudates, cracked lips, and red eyes. He has diffuse lymphadenopathy and slight hepatomegaly. He is very irritable.


ESR: 80 mm/hrRBC: mild anemiaHgb: 1.1 gm/dlWBC: 17,000/mm3Platelet count: 450,000/mm3Urinalysis: some WBCs but no bacteria or proteinuriaEchocardiography: normalSpinal fluid analysis: a few lymphocytes but no organisms on gram stain

QuestionWhich of the following is the most appropriate treatment?

A. IVIG 2 gm/kg over 12 hours

B. Aspirin 325 mg/day

C. Prednisone 2 mg/kg/day

D. Ibuprofen 40mg/kg /day

E. Infliximab 5 mg/kg single dose

Correct AnswerA

Answer RationaleThe clinical description is classic for Kawasaki disease. Although staph toxic shock syndrome and EBV and CMV infections can sometimes present in a similar way, echocardiographic changes are often later in the course.

When there is a clinical index of suspicion for Kawasaki disease, the recommended treatment is high dose IVIG over 12 hours. This is most effective early in the course, before periungual desquamation or echocardiographic changes have occurred. Corticosteroids may be used if IVIG has failed; aspirin (not ibuprofen) is recommended as an antiplatelet agent. Studies are ongoing to determine effectiveness of anti-TNF agents in Kawasaki disease; their effectiveness in treatment is not yet established.

Question Number: 10

CaseA 35-year-old man has had the gradual onset of pain and stiffness in his legs when he jogs one-half mile on a treadmill. He has cramps in his calves and hamstrings toward the end of the half-mile jog. The pain, stiffness, and cramps resolve with rest. Physical examination shows 4/5 hip flexor muscle strength. Serum creatine kinase level is 324 U/L. Electromyography reveals low amplitude motor unit action potentials. Ischemic forearm muscle testing produces the following results:

Venous Blood lactate (mg/dL) NH3 (µg/dL)At rest 8 20Two minutes after exercise 9 90

QuestionWhich of the following deficiencies is the most likely diagnosis?

A. Acid maltase

B. Carnitine palmitoyltransferase II

C. Myoadenylate deaminase

D. Myophosphorylase

E. Phosphorylase b kinase

Correct AnswerD

Answer RationaleIn phosphorylase b kinase deficiency there is a normal or only partially impaired rise in venous lactate with exercise. Ischemic forearm testing reveals a rise in blood lactate with impaired increase in ammonia levels in myoadenylate deaminase deficiency. In acid maltase deficiency the ischemic forearm muscle test is normal. CPT II deficiency usually begins in childhood with exercise-induced myalgia. The ischemic forearm test is normal in CPT II. McArdle’s disease, characterized by myophosphorylase deficiency, is the only choice associated with a flat venous lactate response to ischemic forearm exercise. Note that testing of muscle biopsy samples has in recent years become a precise diagnostic tool to confirm enzyme defects in metabolic muscle disease.

Question Number: 11

CaseA 73-year-old woman who has had deforming rheumatoid arthritis for the

past 20 years now has persistent neutropenia. She has been living in an assisted care facility for the past 3 years. Despite bilateral knee arthroplasties, her mobility and ability to live independently have been progressively impaired by arthritis of her ankles, feet, wrists, and hands. Past therapies have included parenteral gold, penicillamine, hydroxychloroquine, methotrexate, and etanercept. At the time of admission to the facility, her WBC was 3200/mm3 and her physician chose to maintain her on prednisone 7.5 mg qAM alone for her rheumatoid arthritis. Her only infection was a urinary tract infection 3 months ago treated with oral ciprofloxacin. Other than the 10-day course of ciprofloxacin, she has not been on any new medications for the past 12 months.

WBC: 1800/mm3 (25% neutrophils, 65% lymphocytes)Hgb: 10.3 gm/dlPeripheral smear: paucity of neutrophils, occasional large lymphocyte with pale blue cytoplasm and azurophilic granules

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

A. Abdominal ultrasonography to measure spleen size

B. Flow cytometry of peripheral blood

C. Test for cyclic citrullinated peptide (CCP) antibodies

D. Granulocyte antibody test

E. Test for antinuclear antibodies (ANA)

Correct AnswerB

Answer RationaleThe leading diagnostic considerations for this patient are Felty’s syndrome and large granular lymphocyte (LGL) syndrome. The LGL syndrome is a clonal disorder of cytotoxic T-lymphocytes and has been classified as leukemia, even though it usually has an indolent clinical course. About 25% of patients with LGL syndrome have rheumatoid arthritis. The demonstration of a clonal population of CD3+, CD8+, CD16+, CD57+ T cells with flow cytometry of the peripheral blood would serve to establish the diagnosis of LGL syndrome. Splenomegaly and granulocyte antibodies are common to both disorders. The histopathology of the bone marrow does not usually differentiate the two.

Question Number: 12

CaseA 44-year-old woman with a 20-year history of lupus undergoes follow-up evaluation. Initial manifestations included photosensitivity, small joint arthritis, fatigue with a positive ANA, dsDNA, and leukopenia. Her symptoms responded to anti-inflammatory agents and hydroxychloroquine, which she

continues to take. She feels reasonably well but has noticed some difficulty climbing stairs. Findings on physical examination are normal. CPK and TSH levels are normal, and there is no evidence of lupus activity on serological studies. She returns 1 month later, and proximal weakness affecting the hip flexors is noted.


CBC: NormalESR: 33 mm/hrCK: 250 U/LdsDNA: 26 IUTSH: 4.9 µU/mlElectrolytes: Potassium 3.4 mmoles/LMg: Normal Ca: Normal

QuestionWhich of the following is the most likely cause of the patient’s proximal muscle weakness?

A. Inclusion body myositis

B. Lupus flare

C. Hydroxychloroquine myopathy

D. Hypothyroidism

E. Hypokalemia

Correct AnswerC

Answer RationaleAntimalarial therapy is commonly used in patients with lupus. Side effects of therapy include irreversible retinal toxicity, rash, and gastrointestinal disturbance. A less common and under recognized complication of therapy is myopathy, which does not appear to correspond with the dose used. The pathogenesis of this myopathy is thought to be related to accumulation of hydroxychloroquine in lysosomes, which then inhibit lysosomal enzymes. It is a reversible myopathy, and thus important to recognize. Inclusion body myositis is more common in men, and primarily affects the distal muscles. There is nothing to suggest a lupus flare, and although the TSH level is at the high end of normal, hypothyroidism is unlikely to explain the weakness.

Question Number: 13

CaseAn 86-year-old man was sent to the hospital from an Alzheimer’s unit 2 days ago because of a 2-day history of a swollen, red, warm, and tender elbow. He has a temperature of 100.9 degrees F (38.27 degrees C). On admission, WBC was 12,500/mm3 with 60% PMN, and chest radiograph showed mild right lower lobe atelectasis. His elbow was aspirated and 4 cc of cloudy fluid were obtained. Synovial fluid analysis showed cloudy fluid with a WBC of 95,000/mm3. Gram stain was negative, and the fluid was sent for culture. The patient was started on an intravenous antibiotic to cover gram-positive bacteria for presumed septic arthritis.

He is now confused, but his mental status is reported to be his usual. His temperature is 99.6 degrees F (37.55 degrees C). His elbow is held in a flexed position and is slightly swollen in the area between the olecranon and lateral epicondyle. It is minimally erythematous, warm, and tender. He resists any attempt to move the elbow. Findings on examination of the other joints are normal. CBC now has a WBC of 10,000/mm3. Radiograph of the elbow shows only soft tissue swelling. His synovial fluid and blood cultures have no growth. Sputum gram stain shows a few gram-positive cocci in pairs but grows out mixed flora.


A. Surgical open drainage of the joint

B. Continue present antibiotic regimen

C. Aspirate the elbow and look for crystals

D. Add another antibiotic to cover gram-negative organisms

Correct AnswerC

Answer RationaleCPPD crystals can elicit an intense inflammatory response in a single joint that may mimic septic arthritis. It is appropriate to start antibiotics in this clinical setting, but when cultures are negative, one should suspect a crystal-induced process. Open drainage is appropriate in septic arthritis, if the joint effusion cannot be controlled by percutaneous aspiration.

Question Number: 14

CaseA 4-year-old girl has recent onset of rash over her knuckles and knees. Nailfold capillaroscopy is shown in Figure 1 below.

Figure

QuestionThe nailfold changes place this patient at highest risk for which of the following?

A. Calcinosis

B. Pulmonary hemorrhage

C. Retinal changes

D. Gastrointestinal vasculitis

E. Corticosteroid resistance

Correct AnswerD

Answer RationaleCalcinosis occurs late in disease and mainly in patients who have had prolonged disease activity.

Both pulmonary hemorrhage and retinal changes on the basis of vasculitis are rare in dermatomyositis, but can occur as a part of the vasculitic picture. However, nailfold capillary changes are a good predictor of risk for gastrointestinal vasculitis, the most common vasculitic complication in juvenile dermatomyositis.

Corticosteroid resistance or dependence does not correlate with nailfold changes.

Educational ObjectiveTo recognize juvenile dermatomyositis and the association of gastrointestinal vasculitis in children with dermatomyositis and nailfold capillary changes.

Question Number: 15

CaseA 65-year-old woman with osteoporosis sustains her second thoracic vertebral compression fracture within the past 12 months despite ongoing therapy with alendronate for the past 3 years. She has routinely taken calcium and vitamin D supplementation ever since osteoporosis was first diagnosed at age 58 years.

QuestionWhich of the following treatment regimens is most likely to be effective in improving this patient’s bone mineral density over the next 3 years?

A. Continue alendronate therapy alone

B. Continue alendronate and administer daily PTH (1-34) concurrently for the next 18 months, after which both drugs are discontinued

C. Continue alendronate and administer daily PTH (1-34) concurrently for the next 18 months, after which alendronate alone is continued

D. Discontinue alendronate therapy and administer daily PTH (1-34) for the next 18 months

Correct AnswerC

Answer RationaleCosman et al (2005) randomized 126 women who had been on alendronate for at least 1 year to continue alendronate and to receive daily PTH (1-34), cyclic (3 month on/3 month off) PTH (1-34), or alendronate alone. Spine bone mineral density increased 6.1% in the daily PTH (1-34) and 5.4% in the cyclic PTH (1-34) groups, but remained unchanged in the alendronate alone group. In a study by Black et al (2005), patients originally randomized to 1 year of treatment with PTH (1-84) were subsequently randomized to receive alendronate or placebo. Lumbar spine bone mineral density continued to increase in both groups in the first year following discontinuation of PTH (1-84), but continued to rise in the second year only in the alendronate-treated patients. In patients who have never been treated with alendronate, there is no clear evidence that the combination of PTH with alendronate provides a synergistic or additive effect on bone mineral density as compared to PTH alone (Black et al, 2003). These studies thus support the conclusion that this patient would have the greatest increase in bone mineral density at the end

of 3 years if she receivs PTH in addition to alendronate for the first 18 months, and then alendronate alone after the discontinuation of PTH.

Question Number: 16

CaseA 61-year-old man was recently admitted to the medical service for management of congestive heart failure. While hospitalized, he experiences acute and spontaneous onset of right knee pain and swelling.

He has a longstanding history of seronegative nodular rheumatoid arthritis that has been refractory to disease-modifying anti-rheumatic drugs (DMARDs), including hydroxychloroquine and methotrexate, but responsive to brief courses of prednisone.

On physical examination, his fingers are deformed with nodules at the proximal but not distal IP joints (see figure below). He has bilateral, cool of the olecranon bursa. His right knee is visibly swollen, erythematous, and warm to the touch and has a ballotable effusion.

Figure

QuestionWhich of the following is the most appropriate course of action?

A. Obtain a radiograph of the inflamed knee

B. Perform arthrocentesis on the inflamed knee

C. Start a nonsteroidal anti-inflammatory drug (NSAID)

D. Start “pulse” corticosteroids

Correct AnswerB

Answer RationaleAlthough the patient carries the diagnosis of “nodular rheumatoid arthritis” and is already being treated with disease-modifying agents, a patient with

rheumatoid nodules would be expected to be seropositive for rheumatoid factor. This merits a reassessment of his diagnosis and management.

The most appropriate course of action is to perform arthrocentesis to assist in both reevaluating the underlying diagnosis and in determining why this joint is flaring out of proportion to the other joints in what is normally a symmetric polyarthritis–rheumatoid arthritis.

Radiography would be useful in ruling out a bony injury if there were a history of trauma. Instead, the patient’s symptoms developed spontaneously and only after admission. NSAIDs and/or pulse corticosteroids are effective interventions for the acute management of many inflammatory arthritides, but their salt- and fluid-retentive effects could be deleterious to the patient because he has congestive heart failure.

Question Number: 17

CaseA 42-year-old woman with longstanding lupus treated with low-dose corticosteroids and hydroxychloroquine has been doing well for many years. She presented at age 18 with severe Raynaud’s phenomenon and finger necrosis that was treated with IV cyclophosphamide. Other manifestations included chronic thrombocytopenia and pulmonary embolus secondary to anti-phospholipid antibodies. She has never had cardiac or kidney involvement. The patient would like to stop taking hydroxychloroquine because she has been doing so well and finds the yearly eye examinations onerous.

QuestionWhich of the following is most appropriate advice to the patient at this time?

A. Stop the annual eye examinations

B. Discontinue the hydroxychloroquine

C. Continue the hydroxychloroquine

D. Discontinue visual field testing at her annual eye examinations

E. Increase the dose of corticosteroids

Correct AnswerC

Answer RationaleHydroxychloroquine is a useful treatment for the cutaneous and musculoskeletal manifestations of lupus. It has little effect upon nephritis or other major organ involvement. Assays are available to assess serum concentrations of hydroxychloroquine, as low levels have been shown to be

associated with increased disease activity. Irreversible retinal toxicity is rare but occurs with greater frequency the longer the drug is used. The permanent visual damage results from binding of the drug to melanin. The recommended dose is not greater than 6.5 mg/kg. Discontinuation of hydroxychloroquine has been associated with an increased risk of flares. Eye examinations should be continued while the patient takes antimalarial medications, especially if the patient has been taking them for many years. A full visual field and color test is required to optimally track the retinal effects of hydroxychloroquine. Increasing steroids in lieu of taking hydroxychloroquine increases the likelihood of corticosteroid-related complications; corticosteroid use should be kept at a minimum.

Question Number: 18

CaseA 66-year-old woman who has a history of corticosteroid-dependent chronic obstructive pulmonary disease and nondisplaced talus fracture 1 year ago now has burning, aching, and numbness of the sole of the right foot and toes. The discomfort is worse at night and improves with ambulation.

QuestionCompression of which of the following nerves is the most likely cause of this patient’s discomfort?

A. Anterior tibial nerve

B. Deep peroneal nerve

C. Posterior tibial nerve

D. Superficial peroneal nerve

E. Common peroneal nerve

Correct AnswerC

Answer RationaleThis patient has tarsal tunnel syndrome marked by posterior tibial nerve compression. Foot trauma including talus or calcaneal fracture predisposes to the development of this condition. Deep peroneal nerve (or anterior tibial nerve) compression causes paresthesias and numbness of the dorsum of the foot. Note that the anterior tibial nerve is now more commonly called the deep peroneal nerve. Superficial peroneal nerve compression causes paresthesias and numbness over the dorsolateral foot and worsens with exercise. Common peroneal nerve compression causes foot drop.

Question Number: 19

CaseA 23-year-old man sustained multiple toe fractures while playing soccer four months ago. Since cast removal, he has had pain in the lower extremities and arthralgias. Past medical history reveals a clavicle fracture at age 3 years, finger fractures while playing volleyball at age 9 years, and foot fractures at age 12 years when he kicked a can. Physical examination shows a thin man who is slightly nervous and sweating. His height is 5 feet 5 inches and has normal dentition and no skeletal deformities. All MCP, ankle, and elbow joints go beyond the normal range of motion. Findings on examination of the skin and heart are normal. The sclerae are pale blue, which he says have always been like that, and there is no family history of this finding. He wears hearing aids.


CBC: normalAlkaline phosphatase: 55 U/LVit D: 23 ng/mL (range: 9.0-37.6 ng/mL)Testosterone: normal

QuestionWhich of the following studies is the most appropriate next step?

A. Skin fibroblast culture for collagen 1 alpha 1 mRNA production

B. Bone densitometry

C. COLA1 gene sequencing

D. Quantitative CT scans

E. Histomorphometric analysis of bone

Correct AnswerB

Answer RationaleThis is a case of type I ostegenesis imperfecta (OI), a rare inherited connective tissue disorder with many phenotypic presentations. The cause of all cases of OI has not been definitely established. Although mutations in one or the other of two genes which code for proteins that combine to form type I collagen (COL1A1 and COL1A2), account for many of the qualitative and quantitative defects in type I collagen seen in OI, its clinical manifestations vary substantially within families. One member may be quite affected clinically, whereas another member with the same mutation may enjoy normal function. Skin biopsy for analysis if type I collagen genes and/or testing of genomic DNA for mutations in COL1A1 and COL1A2 may be a helpful in diagnosis especially in those with subtle clinical manifestations. However, normal results of these tests do not exclude the diagnosis. Identifying a mutation in a particular gene does not necessarily

result in a clear clinical diagnosis for this reason the clinical diagnosis of OI is based on the signs and symptoms as described in the above case. Mild (type I) OI, is the least severe form. Individuals with type I OI may have few or no fractures before puberty or numerous fractures throughout their lives. Deformity is minimal and stature is usually normal. Individuals with type I OI usually do not begin to have fractures until they begin toddling or walking. The most frequently involved bones are the long bones of the arms and legs, ribs, and the small bones of the hands and feet. Clinical manifestations of OI include excess or atypical fractures (brittle bones), short stature, scoliosis, basilar skull deformities, which may cause nerve compression or other neurologic symptoms, blue sclerae, hearing loss, opalescent teeth that wear quickly (dentinogenesis imperfecta) increased laxity of the ligaments and skin with easy bruiseability. Measurement of BMD by dual-energy x-ray absorptiometry (DEXA) at any age discloses a significant decrease in bone mass. This is the case for mild disease, in which BMD is reported to be 76 percent of that of an age-matched control in the spine and 71 percent of control in the femoral neck. T scores (i.e., standard deviation from the young-adult mean BMD) are frequently in the range of -2.5 to -4.0 at the lumbar spine or proximal femur. Although bone biopsy is indicate when there is excessive skeletal fragility under unusual circumstances and in this case he had a classic clinical picture of IO with multiple fractures at young age, blue sclerae, short stature, and hearing loss.

Question Number: 20

CaseA 23-year-old woman has a 4-year history of polymyositis. At the onset of disease, she had a rash on her face and CK level was 35,000 U/L. EMG was consistent with inflammatory myopathy. A biopsy of the deltoid muscle was consistent with polymyositis. She was subsequently treated with numerous drugs including high-dose prednisone, methotrexate, mycophenolate mofetil, and infliximab. She had an anaphylactic reaction to azathioprine. She has also received several courses of IVIG. None of these treatments have shown significant benefits although the CK levels are now lower, in the range of 8,000-10,000 U/L.

She now has significant difficulties with daily activities, requiring help for dressing and ambulation from her mother who lives with her. At times she uses a scooter. Social history is negative for use of illicit drugs, tobacco, and alcohol. Her family history is negative for autoimmune disease; she is an only child. On examination, she is overweight, has cushingoid features, and is without rash or nail abnormalities. Muscle strength is 2-3/5 in all four extremities, worst in proximal muscles of both legs. An MRI scan using a STIR sequence shows increased signal intensity in muscles of the quadriceps, consistent with the presence of inflammation.


CK: 8,000 U/L (normal range 40-150)Creatinine: 0.6 mg/dL(0.6-1.5)Blood glucose (fasting): 160 mg/dL (70-110)Westergren ESR: 40 mm/hr (1-30)Thyroid-stimulating hormone: 2 uU/ml (0.5-5.0)ANA: negativeJo-1 antibody: negativeUrinalysis: negative for protein, blood, and leukocytes

The decision is made to reevaluate her disease status and obtain another muscle biopsy.

QuestionWhich of the following is the most likely finding on the repeat biopsy?

A. Inclusion body myositis

B. Dermatomyositis

C. Limb girdle muscular dystrophy

D. Polymyositis

E. Diabetic vasculopathy

Correct AnswerC

Answer RationaleThe mean age of onset of limb girdle muscular dystrophy (LGMD) Type 2B, a dysferlinopathy, is 19 years of age. Proximal lower extremity weakness is a common initial finding, and unlike some types of juvenile dystrophies, hypertrophy of calf muscles usually does not occur. Truncal weakness can be marked, and this affects balance and gait making walking difficult. MRI STIR studies in LGMD can show increased signal intensity that is not readily distinguished from the pattern seen in inflammatory myopathies. For these reasons, this disorder can be misdiagnosed as polymyositis. However, the muscle biopsy shows defective staining for dysferlin, a muscle membrane protein, and this feature distinguishes the histologic changes from those seen with polymyositis.

Another clue that this case may not be PM is the very high initial CK level, more than 100 times greater than the upper limit of normal, which is uncommon in the inflammatory myositis syndromes. Chronic juvenile DM is typically associated with more prominent cutaneous features than those described here and frequently also has vasculitic changes and prominent soft tissue calcifications, which are not present in this patient. Furthermore, this young woman is not in the demographic category typical for IBM, which is

generally observed in middle-aged or older men. Diabetes mellitus can alter blood flow in muscles and cause MRI changes, but this is usually associated with longstanding disease, which this patient does not have.

Question Number: 21

CaseA 46-year-old woman has had a 4-month history of recurring confluent erythematous macules over the sun-exposed areas of the forearms, anterior torso, face, and neck that are occasionally associated with superficial ulceration. She has not had associated mouth ulcers, sicca symptoms, xerostomia, or joint problems. Evaluation at the time her symptoms began was remarkable only for the rash and an otherwise very fair complexion; serology studies were notable for positive ANA at a titer of 1:640 (speckled pattern) and anti-SSA/Ro antibodies in a significant elevated titer. She was initially advised to use a high SPF sunscreen and was prescribed hydroxychloroquine 400 mg daily and prednisone 40 mg daily. The rash promptly responded to corticosteroids but has flared each time the prednisone is tapered below 20 mg/day. Other than persistent leucopenia (WBC 2,200/mm3), other blood counts, chemistry profile, and urinalysis have been repeatedly normal.

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

A. Add pulse intravenous cyclophosphamide 750 mg/m2

B. Add azathioprine 2 mg/kg/day

C. Add dapsone 50 mg/day

D. Add quinacrine 100 mg/day

E. Add thalidomide 50 mg/day

Correct AnswerD

Answer RationaleCutaneous manifestations of lupus are quite variable, manifesting as erythematous macular or maculopapular rashes, discoid plaques, bullous lesions, or vasculitis manifest as nonblanching purpura, cutaneous ulcers, or urticarial lesions. While antimalarials are effective for suppressing many of the cutaneous manifestations of lupus and corticosteroids in sufficient dose will usually effect resolution of cutaneous flares, other immunomodulating therapies are often required to suppress cutaneous manifestations of lupus to avoid the toxicity associated with long-term corticosteroid use.

Intravenous pulse cyclophosphamide is effective in managing severe manifestations of SLE but is typically reserved for severe lupus nephritis,

severe CNS manifestations, or refractory severe immune-mediated cytopenias, with other less toxic options employed to manage active skin disease. Azathioprine is often effective as a corticosteroid-sparing therapy for a variety of lupus manifestations, including skin disease. However, concurrent leucopenia as is present in this patient may preclude use of azathioprine in doses sufficient to effectively manage skin disease. In the setting of leucopenia, mycophenolate mofetil might also be considered as an alternative to azathioprine as a corticosteroid-sparing therapy because it impacts predominantly lymphocyte proliferation with minimal impact on granulopoiesis, but the efficacy of mycophenolate in managing cutaneous manifestations of lupus is not well established.

Other alternatives that do not impact granulopoiesis, such as dapsone, addition of a second antimalarial, or thalidomide, are often quite effective in managing cutaneous manifestations of lupus. Dapsone has been shown to be particularly useful in managing cutaneous ulcers in SLE caused by small vessel vasculitis or bullous disease. Persistent mauclopapular eruptions that are not adequately suppressed with hydroxychloroquine often will respond to addition of a second antimalarial such as quinacrine. If this option is used, combined use of these two antimalarials is more effective than either alone and is the concurrent use of both is recommended rather than discontinuing hydroxychloroquine and adding quinacrine. Quinacrine is usually well tolerated although patients with a fair complexion may find it objectionable because of predictable yellow discoloration of the skin with prolonged use. Thalidomide is highly effective in managing mucocutaneous ulcers, as well as refractory erythematous rashes, including the eruption associated with subacute cutaneous lupus, a variant associated with elevated titers of anti-SSA/Ro antibodies.

Question Number: 22

CaseAn 18-year-old woman has 1-year history of progressive proximal muscle weakness. Four months ago, her CK level was 12,000 U/L, a short tau inversion recovery (STIR) MRI showed edema in her proximal thighs, and a muscle biopsy revealed fiber degeneration and regeneration with infiltration of some lymphocytes and phagocytic cells. She was treated with high-dose corticosteroids, methotrexate, and monthly infusions of intravenous immunoglobulins for 3 months. Despite this treatment, muscle strength has continued to deteriorate and CK level is 16,000 U/L.

Her family history reveals that similar symptoms developed 3 years ago in her 26-year-old brother.

QuestionTesting of a repeat muscle biopsy specimen is most likely to show which of the following?

A. Histochemistry revealing fiber type grouping

B. Immunohistochemistry showing absence of dysferlin

C. Trichrome staining revealing ragged red fibers

D. Absence of carnitine palmitoyltransferase (CPT) activity

Correct AnswerB

Answer RationaleThe idiopathic inflammatory myopathies can be confused with other myopathies that may have an inflammatory component, such as muscular dystrophies. Dysferlin deficiency causes limb-girdle muscular dystrophy. This condition is familial with the usual onset between 18 and 20 years of age. CK levels may be 100 times the upper limit of normal. Muscle histology typically shows inflammation. Corticosteroid therapy is not helpful and, in some cases, may make the condition worse.

Fiber type grouping is characterisc of neuropathic processes that involve reinervation.

Ragged red fibers are seen by Trichome staining of muscle in patients with some mitochondrial myopathies. Mitochondrial myopathies may be familial, can present with proximal weakness and elevated CK levels, and will not respond to immunosuppressive therapy. Muscle tissue would not show regeneration or inflammatory cell infiltrate.

This clinical course is not consistent with CPT deficiency. CPT deficiency causes episodic rhabdomyolysis. Between episodes, strength and muscle enzymes are normal.

Question Number: 23

CaseA physician colleague asks for evaluation because he thinks he may have rheumatoid arthritis. He is 55 years old and enjoys excellent health; he is an avid bicyclist. Nine months ago, mild pain and swelling developed in the MCP joint of his right thumb. Two months ago, similar symptoms developed in the PIP joint of the left ring finger. Both sets of symptoms developed without antecedent trauma. There is no morning stiffness. On physical examination, there is mild soft tissue swelling with minimal tenderness of the two symptomatic joints. No other joint abnormalities are present.


ESR: 1 mm/hrC-reactive protein: <0.7 mg/dLRheumatoid factor: 256 IU/ml CCP antibody level: >100

QuestionAccording to the available evidence from clinical trial data, which of the following management approaches is most likely to retard progression of this patient’s arthritis?

A. Prednisone 10 mg po daily

B. Naproxen 500 mg po twice daily

C. Sulfasalazine 1000 mg po bid

D. Etanercept 25 mg subcutaneously twice weekly

E. Methotrexate 15 mg po once weekly

Correct AnswerE

Answer RationaleThis patient does not meet the ACR criteria for rheumatoid arthritis and would thus be classified as having an undifferentiated arthritis. In contrast to early rheumatoid arthritis, there are few clinical trials that have examined therapy for patients with undifferentiated arthritis. The best such study was the PROMPT trial in which 110 patients with undifferentiated arthritis (defined by the 1958 ACR criteria for probable RA) were randomized to receive methotrexate 15 mg weekly or placebo. The dose of methotrexate was increased every 3 months if the Disease Activity Score (DAS) was >2.4. The primary end-point was the fulfillment of the ACR criteria for RA. Undifferentiated arthritis progressed to RA in 22 of the 55 methotrexate-treated patients and in 29 of the 55 placebo-treated patients (p=NS). All of the placebo-treated patients who eventually fulfilled ACR criteria did so within one year, in contrast to only one-half of the methotrexate-treated patients (p=0.04). At the end of 18 months, fewer methotrexate-treated patients showed radiographic progression compared to the placebo-treated patients (p=0.046). Methotrexate was effective in reducing the signs and symptoms of undifferentiated arthritis.

Question Number: 24

CaseA 52-year-old woman is referred for further evaluation of progressive fatigue, dyspnea, and stiffness in her hands. She began to have Raynaud’s symptoms 3 years ago and for the past year has had mild nonproductive cough with increasing exertional dyspnea. For the past 6 months, her fingers have become progressively stiff with impaired grip function. She has not had any

fever, rash, mouth ulcers, dysphagia, or changes in bowel function. Physical examination shows blood pressure 120/86 mmHg, heart rate 100 bpm, and normal respirations and temperature. Sclerodactyly without terminal digital ulcers is present in the upper extremities with mild induration present on the forearms and face. Ocular and oral membranes are notable for pallor but are otherwise normal without evidence of ulceration or xerostomia. Bibasilar crackles are present on chest auscultation but there is no audible pleural or pericardial rub and the pulmonic second heart sound is not accentuated. Other than limitation in extension of the wrists and IP joints of her hands, findings on joint examination are normal. Muscle strength testing is normal.


WBC: 6200/mm3Hct: 24% (MCV 76)Hgb: 7.4 mg/dlPlatelet count: 720,000/mm3Creatinine: 1.6 mg/dlCK: 380 IU/ml (nl<190)AST: normalALT: normalUrinalysis: negative protein, negative blood; 0-2 WBC/hpf, 0-2 RBC/hpf

Chest CT scan: bibasilar interstitial disease, no ground-glass opacities

Echocardiogram: normal LV, RV ejection fraction, mild pericardial effusion, with estimated PA systolic pressure of 43 mmHg

Pulmonary function tests: mild restrictive impairment, DLCO 60% of predicted; DLCO corrected for alveolar volume 86% of predicted.

Renal ultrasonography: normal size kidneys

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

A. Esophagogastroduodenoscopy (EGD)

B. Bone marrow aspiration and biopsy

C. Open-lung biopsy

D. Muscle biopsy

E. Right heart catheterization

Correct AnswerA

Answer RationaleComprehensive management of systemic sclerosis requires attention to complications that may occur in multiple visceral organs including the lungs,

heart, kidneys, and gastrointestinal tract. Significant anemia may occur in patients with systemic sclerosis caused by iron deficiency or renal dysfunction. Chronic gastrointestinal blood loss may occur because of gastrointestinal arteriovenous ectasias (GAVE) that are prevalent in patients with systemic sclerosis or erosive esophagitis arising from chronic reflux. GI blood loss caused by GAVE should be considered in any patient with systemic sclerosis and signs of iron deficiency anemia (microcytic indices, thrombocytosis). Active bleeding can be managed with electrocautery or laser treatment of lesions identified at endoscopy. Other clinicians advocate use of estrogen if not otherwise contraindicated.

It is important to recognize that elevations in creatinine levels may herald impending scleroderma renal crisis, even in the absence of elevated blood pressure, and should prompt institution of treatment with an angiotensin-converting enzyme inhibitor. While anemia resulting from chronic kidney disease may occur as a complication of systemic sclerosis, treatment with erythropoietin preparations should not be instituted until other treatable causes of anemia have been excluded.

Interstitial lung disease in systemic sclerosis causes significant morbidity and is most commonly seen on radiographs as basilar interstitial markings. Alveolitis is reliably detectable by high-resolution CT scan (without contrast). In the absence of demonstrable alveolitis, there are at present no known efficacious treatment interventions for scleroderma-associated ILD and performance of a lung biopsy is seldom required for assessment and management. In the absence of marked impairment of the DLCO corrected for alveolar volume on pulmonary function testing or abnormalities on echocardiography suggestive of pulmonary hypertension such as RV dilation or elevated estimated PA systolic pressure, right heart catheterization would not be indicated.

Elevations in creatine kinase (CK) levels may occur in patients with systemic sclerosis, either because of concomitant hypothyroidism or inflammatory myopathy. If there is no clinical weakness in association with moderate mild to moderate CK elevations, it is not necessary to obtain a muscle biopsy. If there is demonstrable weakness in a pattern consistent with inflammatory myopathy and EMG findings are consistent with a myopathy (as opposed to a primary neuropathic process), it may still be appropriate to proceed with treatment for inflammatory myopathy without obtaining a confirmatory muscle biopsy in patients with established systemic sclerosis. Treatment options other than corticosteroids, such as weekly methotrexate, azathioprine, or IVIG, should be considered due to the increased risk of renal crisis associated with corticosteroids use in patients with systemic sclerosis.

Question Number: 25

CaseA 32-year-old woman has a 2-month history of lower extremity violaceous macules, generalized arthralgias, and asthenia.


HCV RNA: positiveC4: 10 mg/dlCryoglobulins: positiveUrinalysis: normalRheumatoid factor: 180 IU/ml

Skin biopsy of the lower extremity rash reveals leukocytoclastic vasculitis.

QuestionIn addition to prednisone, which of the following is the most appropriate treatment?

A. Peg-Interferon – Ribavirin

B. Plasmapheresis

C. Cyclophosphamide

D. Rituximab

E. Infliximab

Correct AnswerA

Answer RationaleMixed cryoglobulinemia is a systemic vasculitis associated with hepatitis C infection. The mixed cryoglobulinemia syndrome is associated with purpura, arthralgia, and asthenia. There may also be neurologic and renal involvement. Treatment of hepatitis C mixed cryoglobulinemia (HCV-MC) may target the viral load or the B-cell arm of autoimmunity. Peg-Interferon─Ribavirin is the first line therapy. For refractory HCV-associated cryoglobulinemia vasculitis or acute severe life-threatening organ involvement, plasmapheresis, corticosteroids in combination with cytotoxic agent, or Rituximab should be considered as treatment options.

Question Number: 26

CaseA 56-year-old woman has a 4-month progression of 2nd and 4th bilateral DIP deformity. Pruritic nodules on the left 4th DIP joint and right pinna also developed. She has a 2-month history of cough, dyspnea, weight loss, and malaise.

Bilateral radiographs of the hands reveal extensive erosive changes in the bilateral 2nd and 4th DIP joints.

Radiograph of the chest reveal hilar lymph nodes.

CT Scan of the chest reveal nodular pleural thickening, pleural effusions, and mediastinal and hilar lymphadenopathy

Biopsy of the 4th DIP nodule reveals villous, hypertrophic synovitis with plasma cells and multinucleated giant cells


A. Rheumatoid arthritis

B. Relapsing polychondritis

C. Seronegative spondyloarthropathy with peripheral joint involvement

D. Multicentric reticulohistiocytosis

E. Psoriatic arthritis

Correct AnswerD

Answer RationaleMulticentric reticulohistiocytosis is characterized by severe destructive arthritis and cutaneous papulonodular lesions. Histiologic analysis of the cutaneous nodules reveals infiltration of multinucleated giant cells and histiocytes of monocyte/macrophage origin. An associated malignancy has been identified in 15-28% of patients, particularly those with mesothelioma, melanoma, lymphoma, sarcoma, ovarian cancer, and leukemia. Other associations include hyperlipidemia, pregnancy, TB, and autoimmune disease. This patient has an associated mesothelioma. Although the other provided diagnoses could be considered in the differential diagnosis, the biopsy supports the diagnosis of multicentric reticulohistiocytosis

Question Number: 27

CaseA 67-year-old woman has new-onset shoulder pain and stiffness. The symptoms began insidiously 2 months ago. She describes pain and stiffness around her shoulders and thighs, especially worse in the morning on arising. She now has pain in these areas during the night which awakens her routinely. Ibuprofen 400 mg qid provides only short-term relief. Four weeks ago, she was examined by her internist who noted “normal findings.”

Laboratory data at that time included:

CBC: NormalESR: 29 mm/hrCR-P: 0.9 U Rheumatoid factor: negativeANA: 1: 40 AST: NormalALT: NormalCK: NormalCreatinine: NormalAlbumin: NormalTSH: 2.1 UUrinalysis: Normal

Past medical history is notable for hypertension for which she takes atenolol 50 mg daily.

On physical examination now, pain restricts maximal abduction and rotation of the shoulders. Cervical spine range of motion is complete. No synovitis is detected. Motor strength is normal in all extremities. There is no muscle tenderness. There is no cranial tenderness and temporal arteries are easily palpated with normal pulsations.


A. Begin prednisone 15 mg/day

B. Begin amitriptyline 10 mg at bedtime

C. Inject shoulders with intra-articular corticosteroids

D. Discontinue atenolol

E. Begin pregabalin 75 mg/day

Correct AnswerA

Answer RationaleThe clinical presentation of this patient is most consistent with polymyalgia rheumatica (PMR). The onset of bilateral, symmetric, shoulder and pelvic girdle stiffness and nocturnal symptoms in an older patient, with the absence of peripheral joint swelling, is most consistent with this diagnosis. Though markers of inflammation such as ESR and C-RP are generally elevated, they can be normal in about 20 percent of patients. These patients with PMR and normal makers of inflammation have a similar clinical course to patients with elevated markers. Their response to low to moderate doses of corticosteroids is generally dramatic.

MRI and ultrasound studies showed that patients who have PMR with normal or high ESRs have similar inflammatory shoulder lesions. These imaging studies may facilitate the proper diagnosis in patients with the typical proximal symptoms of PMR who also have normal ESRs.

Intra-articular corticosteroids may provide short-term pain relief, but would not be recommended given the strong likelihood of symptom relapse. Increasing the dose of ibuprofen would likely provide little additional benefit and there is no evidence that NSAIDs are effective in managing patients with PMR.

There are no clinical features to suggest fibromyalgia or another soft tissue pain syndrome, thus prescribing amitriptyline or pregabalin is not indicated. Beta-blockers such as atenolol do not cause these pain syndromes and so this medication should not be discontinued.

Question Number: 28

CaseA 73-year-old woman who has had deforming rheumatoid arthritis for the past 20 years now has persistent neutropenia. She has been living in an assisted care facility for the past 3 years. Despite bilateral knee arthroplasties, her mobility and ability to live independently have been progressively impaired by arthritis of her ankles, feet, wrists, and hands. Past therapies have included parenteral gold, penicillamine, hydroxychloroquine, methotrexate, and etanercept. At the time of admission to the facility, her WBC was 3200/mm3 and her physician chose to maintain her on prednisone 7.5 mg qAM alone for her rheumatoid arthritis. Her only infection was a urinary tract infection 3 months ago treated with oral ciprofloxacin. Other than the 10-day course of ciprofloxacin, she has not been on any new medications for the past 12 months.

WBC: 1800/mm3 (25% neutrophils, 65% lymphocytes)Hgb: 10.3 gm/dlPeripheral smear: paucity of neutrophils, occasional large lymphocyte with pale blue cytoplasm and azurophilic granules

Flow cytometry of peripheral blood demonstrates a clonal population of CD3+, CD8+, CD16+, CD57+ T cells

QuestionWhich of the following is the most appropriate initial therapy for this patient?

A. Splenectomy

B. Increased dose of prednisone

C. Infliximab

D. Methotrexate

E. Filgastrim

Correct AnswerD

Answer RationaleThis patient has large granular lymphocyte (LGL) syndrome resulting in an autoimmune neutropenia. The LGL syndrome is a clonal disorder of cytotoxic T-lymphocytes and has been classified as leukemia, even though it has an indolent clinical course. About 25% of patients with LGL syndrome have rheumatoid arthritis. The demonstration of a clonal population of CD3+, CD8+, CD16+, CD57+ T cells with flow cytometry of the peripheral blood served to establish the diagnosis of LGL syndrome.

Methotrexate is considered first line therapy for patients with RA and LGL syndrome. Cyclosporine A may also be of benefit in patients with LGL syndrome. There are no data to indicate that glucocorticoids have lasting benefit in the therapy for these diseases. While splenectomy may result in an immediate improvement in the absolute neutrophil count, the clinical response is usually not lasting. Given the morbidity of a splenectomy, it is usually reserved for patients with active infections that are resistant to treatment or patients with chronic refractory leg ulcers. Myeloid colony-stimulating factors, such as filgastrim, may be used in severe neutropenia while a remission is being induced with immunosuppressive agents. They have the potential for inducing a flare of the rheumatoid arthritis. The therapeutic benefit of TNF antagonists in these disorders has not been established.

Question Number: 29

CaseA 26-year-old man has had myositis for the past 5 years. He first noted weakness a few months after completing 2 years of military service at age 21 years. Initially, he had symmetric weakness confined to his proximal legs, making it difficult for him to climb stairs or arise from a chair. CK level was 4530 U/L, and electromyography showed small amplitude and short duration motor unit action potentials but no spontaneous activity. A biopsy of the right quadriceps muscle showed infiltrates of mononuclear cells around necrotic muscle fibers. Treatment for polymyositis with corticosteroids, methotrexate, and azathioprine did not result in any improvement in muscle strength. Over the past year, isolated weakness of his biceps muscles in both arms has developed. There is no scapular winging and no facial weakness. He has no respiratory muscle weakness.


A. Facioscapulohumeral muscular dystrophy

B. Sarcoglycanopathy

C. Proximal myotonic dystrophy

D. Becker’s dystrophinopathy

E. Dysferlinopathy

Correct AnswerE

Answer RationaleBecker’s muscular dystrophy usually presents with shoulder and pelvic girdle muscle weakness in childhood, but, in contrast to Duchenne’s dystrophy, affected boys remain ambulatory beyond the age of 16 years. Some BMD patients do not develop symptoms until after the age of 30 years and can continue to walk until the seventh decade of life. CK levels are elevated in all patients with BMD.The limb-girdle muscular dystrophies (LGMD) are a heterogeneous group of disorders characterized by progressive proximal muscle weakness which are distinct from the more common X-linked dystrophinopathies. Affected boys and girls usually present in the second or third decade of life with weakness of the proximal leg muscles. Upper limb involvement develops later. The inheritance is autosomal, most frequently recessive. LGMD type 2B (LGMD2B) and Myoshi distal myopathy result from mutations in the dysferlin gene and are known as ‘dysferlinopathies.’ LGMD2B is characterized by weakness and atrophy of the pelvic and shoulder girdle muscles, developing in adolescence or young adulthood and progressing slowly thereafter. Physical activity before onset is normal. Weakness begins in the lower extremities and may not progress to the upper extremities until ten or more years later. Weakness of the biceps muscles generally occurs first in the upper extremities. Some patients may have both proximal and distal muscle weakness at onset. Scapular winging does not occur. LGMD2B may be misdiagnosed as polymyositis because of its onset in adult life, marked elevation of serum CK level, and the presence of inflammatory infiltrates in the muscle biopsy. The sarcoglycanopathies are forms of LGMD (types 2C, D, E, and F) caused respectively by mutations in the , α, ß, and -sarcoglycan genes and primarily affect Caucasians. Onset is most often in early childhood and loss of ambulation generally occurs before the age of 16 years. However, milder phenotypes do occur, with proximal muscle weakness developing in young adulthood. Muscle weakness is initially

detectable in the pelvic girdle, and later involves the shoulders, with scapular winging. Respiratory muscle involvement occurs late in the disease and frequently leads to respiratory failure and death. Serum levels of CK are elevated 10-100x ULN, typically higher than in the other forms of LGMD. Facioscapulohumeral muscular dystrophy (FSHD) is an autosomal dominant disorder that predominantly affects the facial, scapulohumeral, pelvic girdle and anterior tibial muscles. Patients first develop symptoms in the first to fifth decades of life. Facial weakness usually begins first. Medical attention is usually sought after weakness of scapular fixation precludes abduction of the arm beyond 90°. Scapular winging is a prominent feature. The serum CK may be normal or mildly elevated. Proximal myotonic dystrophy, type 2 (DM2) is an autosomal dominant disorder characterized by proximal muscle weakness, muscle pain, cataracts, myotonia, tremors, cardiac disturbances, and hypogonadism. The neck flexors, hip flexors, and triceps muscles are most commonly affected. This disease is distinct from myotonic dystrophy (DM1), having no temporal atrophy, wasting of the sternocleidomastoid muscles, or ptosis and either minimal or no distal weakness. The myotonia is also atypical in DM2, being commonly asymmetric, variable in severity, and intermittent in occurrence. Serum CK levels are elevated 2-4x ULN.

Question Number: 30

CaseA 35-year-old woman has had pain and swelling in her right foot for the past 3 weeks. Two months ago, she tore the medial meniscus and anterior cruciate ligament in her right knee while skiing. These were repaired with arthroscopic surgery. She was hospitalized on the third postoperative day for severe pneumonia. Pulmonary function became compromised and she required mechanical ventilation for 3 days. Total hospitalization was 11 days.

About 3 weeks ago, she began having swelling of her right foot. Over the next few days, the foot became tender and she could not bare weight on it. At first the pain was with use but soon became constant and more severe. She describes the pain as both “lancinating and numb.” Now she is afraid to let anyone or anything touch the foot because of the intense increase in pain that occurs.

Physical examination shows global swelling of the right foot and ankle. The foot, up to the level of the malleoli, is erythematous, warm, and extremely tender. Ankle and subtalar joint motion is diminished. Homan’s sign is positive.

ESR and CBC are normal. Radiograph of the foot shows patchy osteopenia.Triple-phase technetium bone scan shows increased blood flow to the right foot in the early phase and increased bone uptake in the late phase.

QuestionIn addition to physical therapy, which of the following is the most appropriate treatment at this point?

A. Amitriptyline

B. Topical capsaicin

C. Prazocin

D. Alendronate

E. Prednisone

Correct AnswerE

Answer RationaleOral corticosteroids, such as prednisone 25 to 80 mg/daily over a few weeks any then tapering, may be effective in treatment of early complex regional pain syndrome, especially in patients with the classic findings seen on triple-phase technetium bone scan. Those findings include increased blood flow to the affected part in the early phase and increased bone uptake in the late phase.

Topical capsaicin and low-dose mid-evening amitriptyline may be helpful but would not be as beneficial as prednisone. Other agents such as bisphosphonates (alendronate), alpha blockers (proazocin), beta blockers (propanolol) or calcium channel blockers (nifedipine) have been used, but appropriate trials assessing their value have not been performed.

Other treatments that may be employed include pregabulin, clonidine, and sympathertoic nerve blocks. Regardless of the medications used, physical therapy is an essential component of the management.

Question Number: 31

CaseA 30-year-old woman who is at 29 weeks gestation in an uneventful pregnancy is referred for evaluation of problems with both hands. She has noticed that over the past 2 weeks she has had tingling and some numbness in the hands, especially when she is using the phone at work. Over the past week she has been waking at night with the same symptoms. She has not noted swelling in the hands, although she has been retaining some fluid in the legs over the past month. Her job involves extensive computer data entry and she is concerned that she will have problems keeping up with her work assignments. Her past history is unremarkable and her only medications are vitamin and mineral supplements. On examination, she has tenderness of both wrists and compression of the wrists elicits numbness in some of her fingers. There is no swelling of the hand or wrist joints.


Westergren ESR: 35 mm/hr (1-30)Rheumatoid factor: 32 IU/ml (<30)

QuestionWhich of the following recommendations is most appropriate?

A. Advise her to wear wrist splints at night

B. Recommend starting prednisone 10 mg/day

C. Nerve conduction studies

D. Request transfer to non-keyboard job duties.

E. Injection of corticosteroids.

Correct AnswerA

Answer RationaleThis woman has carpal tunnel syndrome related to her pregnancy. The prevalence of carpal tunnel syndrome is increased in the third trimester of pregnancy and it generally goes away after pregnancy. Wrist splints remain the mainstay of conservative therapy. Corticosteroids are not needed for this self-limited syndrome and further nerve conduction testing is also not indicated unless the problem persists after pregnancy. The small elevations in ESR and RF may be related to pregnancy and an inflammatory joint condition like rheumatoid arthritis is unlikely. A recent study suggests that contrary to what many physicians and patients have thought, computer keyboard work may be associated with a lower rather than higher prevalence of carpal tunnel syndrome.

Question Number: 32

CaseA 43-year-old woman who has had Churg-Strauss vasculits for the past 3 years is seen for a follow-up evaluation. Three years ago, she presented with a multisystem disease characterized by asthma, renal insufficiency, and peripheral neuropathy. She was treated with corticosteroids and cyclophosphamide with improvement noted after 6 months of therapy. She has been maintained on intermittent courses of corticosteroids for asthma flares.On physical examination, her blood pressure is 110/60 mmHg, pulse rate is 72 bpm, and respirations are 14/min. On examination of the chest, a few scattered wheezes are noted.Heart sounds are normal and there are no murmurs. Examination of the abdomen shows no organomegaly or masses.

Neurological examination shows intact strength in all extremities and a slight perception of numbness in the toes of the right foot.

Laboratory data include:CBC: normalESR: 23 mm/hrC-RP: 1.6 mg/dLSerum creatinine: 1.7 mg/dlANCA: negativeUrinalysis: 2+ protein, no cells, no casts

The patient is concerned about her prognosis and discussion about this issue is initiated with her.

QuestionOf the following clinical findings for Churg-Strauss vasculits which is the strongest indicator of a poor prognosis in this patient?

A. Cardiac involvement

B. Gastrointestinal disease

C. Serum creatinine concentration >1.6 mg/dL [141 mmol/L]

D. Proteinuria (>1 g/day)

E. Central nervous system involvement

Correct AnswerB

Answer RationaleAlthough the prognosis for patients with Churg-Strauss vasculits (CSS) is unclear, treatment appears to have significantly decreased mortality. Prior to the use of glucocorticoids, for example, the disease was uniformly fatal, with 50 percent of untreated patients dying within 3 months of the onset of vasculitis. In comparison, most modern clinical studies suggest a survival rate of greater than 70 percent at 5 years. Most deaths result from complications of the vasculitic phase of the disease and are most commonly due to cardiac failure and/or myocardial infarction, cerebral hemorrhage, renal failure, gastrointestinal bleeding, or status asthmaticus.The presence or absence of certain clinical features strongly correlates with subsequent mortality. The following five clinical factors appear to have significant prognostic value in patients with CSS:• Cardiac involvement• Gastrointestinal disease• Renal insufficiency (plasma creatinine concentration >1.6 mg/dL [141 mmol/L])• Proteinuria (>1 g/day)

• Central nervous system involvement

Of these, the presence of significant cardiac or gastrointestinal disease appears to be the strongest indicator of poor prognosis. A "five-factor score" based on the above risk factors has been used to predict survival in CSS, and one large series reported the following 5-year mortality figures:• 12 percent when none of the 5 prognostic factors was present• 26 percent when 1 factor was present• 46 percent when 3 or more factors were present

A shorter duration of asthma before the onset of vasculitis also portends a poorer prognosis. The role of ANCA measurements in predicting outcome is not known.

Question Number: 33

CaseA 21-year-old man had an episode of rhabdomyolysis. The patient says that the episode occurred after he tried to start a new exercise program on a warm day. He thinks he was well hydrated the day of the event. He says he has never been good in sports and is mainly a “couch potato.” His favorite hobby is playing video games. Every time he tries to run he gets leg cramps. He could never perform well in high school physical education programs involving aerobic-type exercises. Findings on review of systems are normal. He drinks socially, denies use of drugs or nutritional products, and is a nonsmoker. On physical examination, vital signs are normal and there is no organomegaly. Findings on cardiopulmonary examination are normal. All proximal and distal muscles have normal strength and are nontender. Findings on neurologic examination are normal.


CBC: normalChemistry: normalMuscle biopsy: normal, special stains are pending


A. Acid maltase deficiency

B. Mytochondrial myopathy

C. Polymiositis

D. Carnitine palmitoyltransferase (CPT) deficiency

E. Disferlinopathy

Correct AnswerD

Answer RationaleMetabolic myopathies are conditions that have in common abnormalities in muscle energy metabolism that result in skeletal muscle dysfunction. Primary metabolic myopathies are associated with biochemical defects that affect the ability of the muscle fibers to maintain adequate levels of adenosine triphosphate (ATP). Secondary metabolic myopathies are attributed to various endocrine and electrolyte abnormalities. Primary metabolic myopathies must be considered in patients who present with dynamic symptoms, such as muscle pain, cramps, or myoglobinuria, with exercise, rather than static symptoms, such as fixed weakness of a specific muscle group, which occur with idiopathic inflammatory myopathies and muscular distrophies. This patient most likely has a lipid or glycogen storage disease, both presenting with dynamic symptoms. Although acid maltase deficiency is a glycogen storage disease, the adult form presents with progressive painless limb-girdle muscles and not as the above case. Patients with fatty acid transport defects as in CPT deficiency usually develop symptoms and signs after prolonged exertion (commonly more than 30 minutes). Rhabdomyolysis can occur with prolonged exercise or other factors such as anesthesia or taking some medications. Affected individuals do not show reduced tolerance to brief exercise. At rest and between episodes, both muscle strength and serum CK level are usually normal.

Question Number: 34

CaseA 23-year-old woman with a history of established SLE manifest by photosensitive rash, serositis, and nephritis confirmed on recent biopsy (WHO class IIIb) presents for a routine follow-up visit. At her last visit one month ago, proteinuria and creatinine levels were stable (1+ and 1.0 mg/dl, respectively) with normal urine sediment. At that time, she was advised to taper her prednisone dose from 15 mg/day to 10 mg/day. She reports no new complaints symptoms but says her menstrual period is two weeks delayed. Current medications include hydroxychloroquine 400 mg/day, mycophenolate mofetil 2,000 mg daily, prednisone 10 mg/day, and lisinopril 10 mg/day. Physical examination is notable for blood pressure of 130/92 mmHg and mild erythema over the sun-exposed areas of her face. Oral and ocular membranes appear normal and there are no audible pleural or pericardial rubs. Extremities are notable for 1+ tibial and pedal edema; finding on joint and neurological examinations are normal.


WBC: 4600/mm3Hct: 30%

Platelet count: 160,000/mm3Creatinine: 1.1 mg/dl (stable)Urinalysis: 0-2 WBC, 10-12 RBC/hpfUrine protein: 180 mg/dlUrine creatinine: 160 mg/dlC3: 42 mg/dl [nl>70]; C4: 9 mg/dl [nl >15]Pregnancy test: positive

She wishes to continue with the pregnancy.

QuestionIn addition to increasing the dose of prednisone, which of the following is most appropriate to recommend with regard to managing the lupus at this time?

A. Continue lisinopril and hydroxychloroquine, increase mycophenolate to 3,000 mg/day

B. Stop lisinopril and mycophenolate; continue hydroxychloroquine; begin labetalol

C. Stop lisinopril; continue hydroxychloroquine and mycophenolate; begin labetalol

D. Stop mycophenolate and lisinopril; continue hydroxychloroquine; begin azathioprine and labetalol

E. Stop hydroxychloroquine, mycophenolate and lisinopril; begin labetalol

Correct AnswerD

Answer RationaleIntercurrent pregnancy in the context of active SLE poses significant diagnostic and therapeutic challenges. Patients with a history of lupus nephritis who become pregnant are at increased risk for hypertension and the development of pre-eclampsia. The differentiation of pre-eclampsia from lupus flare is often difficult diagnostically when edema, hypertension, and/or azotemia develop during the late second or third trimester of pregnancy, but the presence of active urine sediment and edema in the very early stages of the pregnancy are more likely attributable to active lupus than to pre-eclampsia.

The presence of active nephritis requires continued aggressive treatment through the course of pregnancy, but the choice of needed immunosuppressive therapy is limited to agents not known to cause deleterious effects on the fetus. ACE inhibitors such as lisinopril are teratogenic and must be discontinued in the context of pregnancy; labetalol is an effective and safe alternative that can be supplemented with vasodilators (such as hydralazine) as needed to manage blood pressure. Mycophenolate has also been shown to be teratogenic and should be discontinued at the onset of pregnancy. Corticosteroids may be continued through the pregnancy in doses required to suppress disease activity, but continued high doses may exacerbate edema and hypertension as well as

promote the development of gestational diabetes. Azathioprine has been used through the course of pregnancy and has not been associated with fetal malformation or birth defects. As such, corticosteroid-sparing therapy with azathioprine to continue aggressive treatment of active SLE nephritis or immune cytopenias is reasonable and may be necessary to manage SLE and pregnancy. Antimalarials have not been shown to induce fetal abnormalities and current recommendations are to continue antimalarial treatment in patients with active SLE who become pregnant.

Question Number: 35

CaseA 35-year-old woman has had pain and swelling in her right foot for the past 3 weeks. Two months ago, she tore the medial meniscus and anterior cruciate ligament in her right knee while skiing. These were repaired with arthroscopic surgery. She was hospitalized on the third postoperative day for severe pneumonia. Pulmonary function became compromised and she required mechanical ventilation for 3 days. Total hospitalization was 11 days.

About 3 weeks ago, she began having swelling of her right foot. Over the next few days, the foot became tender and she could not bare weight on it. At first the pain was with use but soon became constant and more severe. She describes the pain as both “lancinating and numb.” Now she is afraid to let anyone or anything touch the foot because of the intense increase in pain that occurs.

Physical examination shows global swelling of the right foot and ankle. The foot, up to the level of the malleoli, is erythematous, warm, and extremely tender. Ankle and subtalar joint motion is diminished. Homan’s sign is positive.

ESR and CBC are normal. Radiograph of the foot shows patchy osteopenia.

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

A. MRI of the foot and ankle

B. DEXA scan of the foot and ankle

C. Venous Doppler studies of the lower extremities

D. Tecnitium triple-phase bone scan of the foot and ankle

Correct AnswerD

Answer RationaleComplex regional pain syndrome (formerly known as reflex sympathetic dystrophy) may occur spontaneously, but typically follows trauma, surgery,

or any incident that leads to immobilization of an extremity. The involved part is typically distal and becomes swollen and tender, manifesting allodynia (severe pain following a light touch) and hyperpathia (intense pain lasting after the painful stimulus is removed). The extremity can appear red or blue and feel warm or cool. Vasomotor changes may be noted as well. Patchy osteopenia is observed on radiography, often within the first weeks of symptoms.

Findings on triple-phase technitium bone scans are abnormal and consistent with increased regional blood flow in the early phase and pooling and increased bone uptake in the late phase.

MRI would confirm the regional bone loss but add no information. A DEXA scan and venous Doppler studies would not be indicated in this clinical setting.

Question Number: 36

CaseA 22-year-old college student has left knee swelling after playing soccer. He does not recall specific trauma to the affected joint. He denies sexual activity. Review of systems is notable for the lack of antecedent infectious symptoms, visual difficulties, oral ulcers, back pain, diarrhea, or rash. His home is in Connecticut, and deer frequent his back yard. On physical examination, the left knee is large and swollen.

Analysis of aspirated joint fluid reveals WBC 11,000/mm3 with predominant neutrophils, no crystals, and negative bacterial cultures. Lyme titer is markedly positive with a confirmatory Western blot test showing 10 positive bands.

He is treated with doxycycline for 30 days. The follow-up examination shows a persistent effusion and no abnormal neurological manifestations. The oral antibiotic is continued for another 30 days. Intravenous ceftriaxone is prescribed for 1 month after the extended course of oral doxycycline fails to eradicate the effusion. Synovial fluid aspiration now shows persistent inflammatory joint fluid with negative PCR for Borrelia burgdorferi.

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

A. Etanercept

B. Oral doxycycline for 30 days

C. IV ceftriaxone for 30 days

D. IV penicillin for 30 days

E. Hydroxychloroquine

Correct AnswerE

Answer RationaleLyme arthritis is an inflammatory arthritis that primarily affects large joints, especially the knee, and is caused by infection with Borrelia burgdorferi, which is a spirochete transmitted by Ixodid ticks. In most patients, treatment with oral doxycycline for 30 to 60 days eradicates infection and the effusion. In a small percentage of patients, a persistent effusion occurs despite months of oral and intravenous antibiotic therapy. In these patients, the appropriate work-up should include PCR testing of the synovial fluid for Borrelia burgdorferi. If the PCR is positive, repeated courses of antibiotic therapy should be considered. If the PCR is negative, antibiotics are not recommended, and treatment with NSAIDs or hydroxychloroquine is recommended. PCR testing is technically challenging and should thus be performed in experienced laboratories.

Question Number: 37

CaseA 65-year-old woman has swelling in the right elbow. Four months ago, she accidentally bumped it against a wall. She noted immediate pain that resolved within few days but not the swelling. She has no pain, morning stiffness, or limited function. She has a past medical history of hypertension with stage II chronic kidney disease (CKD), takes Lisinopril 10 mg daily, is a nonsmoker and nondrinker, and has not traveled outside the USA. The patient is alert, oriented, and in no distress. Physical examination shows normal vital signs. With the exception of a nontender firm nodular mass on the right olecranon area, findings on the rest of the physical examination are normal.


Creatinine: 2.0 mg/dl (creatinine a year ago was 1.6 mg/dl)CBC: Hgb 11 gm/dl; Hct 34% with normal indicesCalcium: 11 mg/dl Phosphate: 3.5 mg/dl Parathyroid hormone (PTH): 65 pg/mlPTH rp: 1.1 pmol/L (normal < 1.5 pmol/L) 25 OH Vit D: 50 nmol/L1,25 OH Vit D: 65 micromol/L

Angiotensin-converting enzyme: 80 U/LUrinalysis: normal24-hr urinary calcium: 350 mg

Chest (Image 1) and elbow (Image 2) radiographs are shown.

Figure

Figure 1 Figure 2

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

A. Hyperparathyroidism secondary to CKD

B. Ectopic production of PTH

C. Primary hyperparathyroidism

D. Increased production of 1,25 Vit D

E. Myositis ossificans

Correct AnswerD

Answer RationaleMild to severe hypercalcemia and/or hypercalciuria occurs in 10% of patients

with sarcoidosis. The cause of this disordered regulation of calcium is due to endogenous production of active metabolite of Vitamin D by macrophages in the sarcoid granuloma. This extra renal synthesis of 1,25 OH Vit D is not subject to the normal physiologic regulatory influences. This patient has CKD with mild secondary hyperparathyroidism. Her mildly elevated PTH failed to suppress 1,25 Vit D and patients with CKD have decreased production of 1,25 in the kidney, meaning that the production of 1,25 Vit D in this case comes from an extra renal source. Risk factors associated with this problem include widespread disease with index of active disease activity like increased ACE levels and diffuse infiltrative pulmonary disease.

RHEUMATOLOGY MCQS- 2008 1-37

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