A
B
C
D
100

A 51-year-old woman is evaluated at a follow-up visit. Sjögren syndrome was diagnosed 3 years ago. For the past 4 weeks she has experienced arthralgia, unintentional weight loss, fever, and drenching night sweats. She has hypothyroidism. Current medications are levothyroxine, artificial tears, and ophthalmic cyclosporine suspension.

On physical examination, vital signs are normal. Parotid glands are enlarged, and the oropharynx is dry. Lungs are clear to auscultation. There are no skin lesions or evidence of joint synovitis.

Laboratory studies:

Erythrocyte sedimentation rate 

73 mm/h

Hemoglobin 

10.2 g/dL (102 g/L)

C3  complement

102 mg/dL (1020 mg/L)

C4  complement

8 mg/dL (80 mg/L)

Thyroid-stimulating hormone 

3.0 μU/mL (3.0 mU/L)

Rheumatoid factor 

442 U/mL (442 kU/L)

Anti–cyclic citrullinated peptide antibodies

Negative

Anti-La/SSB antibodies

Negative

Anti-Ro/SSA antibodies

>8 U/L (normal, <1 U/L)

Urinalysis

0-1+ protein; no erythrocytes, leukocytes, or casts

Immunofixation studies show a monoclonal IgM gammopathy (κ type).

Which of the following conditions is most likely responsible for her recent symptoms?

AL amyloidosis

Hypothyroidism

Lymphoma

Rheumatoid arthritis

100

A 42-year-old woman is evaluated for increasingly severe pain and swelling in both hands for the last 6 months. She has morning stiffness that lasts 1 hour. Current medication is ibuprofen.

On physical examination, vital signs are normal. Joint examination reveals bilateral swelling and tenderness of the second and third metacarpophalangeal joints and third proximal interphalangeal joint.

Laboratory studies:

Hemoglobin 

10.8 g/dL (108 g/L)

Rheumatoid factor

Positive

Anti–cyclic citrullinated peptide antibodies

Positive

Antinuclear antibodies

Negative

Which of the following is the most appropriate test to perform next?

ANCA

Anti–double-stranded DNA antibodies

MRI of hands

Plain radiography of hands

100

A 55-year-old woman is evaluated for a 5-day history of increasing pain, swelling, and warmth in the left knee. She has a 30-year history of rheumatoid arthritis. Current medications are etanercept, prednisone, methotrexate, folic acid, and meloxicam.

On physical examination, temperature is 37.5 °C (99.5 °F); other vital signs are normal. Joint examination of the hands reveals nonpainful deformities and restricted movement characteristic of advanced but quiescent rheumatoid arthritis. The left knee exhibits warmth and a moderate effusion without erythema; flexion is restricted because of pain. The right knee shows bony hypertrophy.

Laboratory evaluation shows an erythrocyte sedimentation rate  of 70 mm/h and leukocyte count  of 13,500/μL (13.5 × 109/L).

Which of the following is the most appropriate diagnostic test to perform next?

Serum procalcitonin

Synovial biopsy

Synovial fluid analysis

C-reactive protein

100

A 40-year-old man is hospitalized for acute pericarditis. He also reports pain in the wrists, ankles, and knees for 1 week but no other symptoms. He has otherwise been well and takes no medications.

On physical examination, temperature is 38.0 °C (100.4 °F); the remainder of the vital signs are normal. A pericardial friction rub is present. The wrists, knees, and ankles are tender. There are small bilateral wrist and knee effusions. The remainder of the examination is unremarkable.

Laboratory studies:

Hematocrit 

30%

Leukocyte count 

4000/μL (4.0 × 109/L), with lymphopenia

Platelet count 

72,000/μL (72 × 109/L)

C-reactive protein 

5.5 mg/dL (55 mg/L)

Creatinine 

1.4 mg/dL (123.8 μmol/L)

Urinalysis

1+ protein, 1+ blood; 2-3 erythrocytes; no leukocytes; no casts

Which of the following is the most likely cause of this patient's pericarditis?

Adult-onset Still disease

Coxsackievirus infection

idiopathic pericarditis

Systemic lupus erythematosus

200

An 18-year-old woman is evaluated for a 1-week history of fever and diffuse myalgia, and pain and swelling in the hands and knees that have progressed over the last month. She also has acne. Current medications are minocycline, topical tretinoin, and an oral contraceptive.

On physical examination, temperature is 38.3 °C (100.9 °F); other vital signs are normal. She has synovitis in her hands and wrists and inflammatory acne on the face and upper back.

Laboratory studies:

Complete blood count

Normal

Erythrocyte sedimentation rate 

36 mm/h

Creatinine

Normal

Anti–cyclic citrullinated peptide antibodies

Negative

Antinuclear antibodies

1:320 (speckled pattern)

ANCA

Positive (p-ANCA pattern)

Rheumatoid factor

Negative

Urinalysis

Normal

Test results for antihistone and other specific autoantibodies are negative, and serum complement levels are normal.

Which of the following is the most likely diagnosis?

Drug-induced lupus erythematosus

Granulomatosis with polyangiitis

Rheumatoid arthritis

Systemic lupus erythematosus

200


A 30-year-old woman is evaluated for a 2-month history of skin changes, primarily on her chest and arms. She has no other symptoms. Her only medication is an oral contraceptive.

On physical examination, vital signs are normal. The rash on her chest is shown.



There is no evident scarring or lesions in the scalp or ears, hair loss, or joint swelling. The remainder of the examination is normal.

Laboratory evaluation reveals an antinuclear antibody titer of 1:640 with speckled pattern; result for anti-Ro/SSA antibody is positive.

Which of the following is the most likely diagnosis?

Acute cutaneous lupus erythematosus

Cutaneous leukocytoclastic vasculitis

Discoid lupus erythematosus

Subacute cutaneous lupus erythematosus

200

A 30-year-old woman is seen for preconception counseling. Three years ago, she was diagnosed with systemic lupus erythematosus and nephritis. She responded rapidly to induction therapy with combination immunosuppressants and has been maintained on mycophenolate mofetil and hydroxychloroquine for the past 2 years. An intrauterine device is used for contraception.

Laboratory evaluation is consistent with quiescent disease, and there is no evidence of kidney dysfunction.

Which of the following is the most appropriate preconception management?

Advise against pregnancy

Continue current medications

Discontinue hydroxychloroquine and mycophenolate mofetil; add prednisone

Discontinue mycophenolate mofetil; add azathioprine

200

A 58-year-old woman is evaluated for a 4-month history of diarrhea and weight loss. Diffuse cutaneous systemic sclerosis was diagnosed 10 years ago. She reports explosive diarrhea soon after eating a meal. She has lost 5 kg (11 lb) since the symptoms began. She also has Raynaud phenomenon and gastroesophageal reflux disease controlled with pantoprazole once a day. Current medications are sustained-release nifedipine and pantoprazole.

On physical examination, vital signs are normal. Skin changes consistent with diffuse cutaneous systemic sclerosis are present. Findings on the abdominal examination are normal.

Which of the following is the most appropriate treatment?

Cholestyramine

Ciprofloxacin

Increase in pantoprazole dosage

Loperamide

300

A 37-year-old woman is evaluated for a 1-year history of widespread joint and muscle pain, fatigue, poor sleep, and difficulty focusing. She also has irritable bowel syndrome and migraine headaches. Current medications are sumatriptan and topiramate.

On physical examination, vital signs are normal. No rash is present on the face or extremities. Joint examination shows normal range of motion and no joint swelling. Most soft tissue is tender to light palpation. Muscle strength is normal.

Laboratory evaluation shows a normal complete blood count, serum thyroid-stimulating hormone level, and urinalysis as well as an erythrocyte sedimentation rate  of 20 mm/h.

Which of the following is the most likely diagnosis?

Fibromyalgia

Generalized osteoarthritis

Polymyalgia rheumatica

Rheumatoid arthritis

Systemic lupus erythematosus

300

A 78-year-old woman is evaluated for constant bilateral headache of 1 week's duration. Two months ago, she was diagnosed with polymyalgia rheumatica. Symptoms were relieved with prednisone, 15 mg/d. Headache appeared shortly after the dosage was tapered to 5 mg/d. She also has experienced new-onset intermittent muscular jaw discomfort with chewing.

On physical examination, vital signs are normal. The scalp and temporal arteries are not tender to palpation. No bruit is heard over the great vessels; temporal artery pulses are intact.

Laboratory evaluation shows a blood C-reactive protein  level of 12.8 mg/dL (128 mg/L).

Which of the following is the most likely diagnosis?

Giant cell arteritis

Granulomatosis with polyangiitis

Microscopic polyangiitis

Polyarteritis nodosa

300

A 60-year-old man is evaluated in follow-up after an episode of podagra, which was treated with colchicine. This was his first gout flare. He also has hypertension and hyperlipidemia. Current medications are hydrochlorothiazide and atorvastatin.

On physical examination, blood pressure and other findings are normal.

Laboratory evaluation reveals a serum urate  level of 9.0 mg/dL (0.53 mmol/L).

Which of the following is the most appropriate treatment?

Start allopurinol

Start vitamin C

Stop atorvastatin and start fenofibrateD

Stop hydrochlorothiazide and start losartan

300

A 29-year-old man is evaluated after an episode of anterior uveitis. He has 2-year history of low back stiffness at night and in the morning that improves with activity. Current medication is meloxicam as needed.

On physical examination, he cannot touch his toes, and there is flattening of the normal lumbar lordosis. Internal rotation and flexion elicit pain in the right hip; range of motion of the right hip is diminished. Flexion, external rotation, and abduction of the hips cause pain in the right hip and low back. The remainder of the examination is unremarkable.

Which of the following is the most appropriate diagnostic test to perform next?

Anteroposterior radiography of pelvis

BAntinuclear antibody assayC

HLA-B27 testing

Sacroiliac joint MRI

400

A 72-year-old man is evaluated for chronic right knee pain with morning stiffness lasting 15 minutes. He is experiencing increased difficulty walking because of knee pain. He has no other medical problems other than overweight. His only medication is acetaminophen, which provides minimal pain relief.

On physical examination, BMI is 34. There is bony hypertrophy of the knee without effusion or signs of inflammation. He has moderate pain with full knee flexion but no restriction of motion. The remainder of the examination is noncontributory.

Topical diclofenac is prescribed.

Which of the following is the most appropriate additional treatment?

Exercise and weight loss

Massage therapy

Mobilization/manipulation and passive range of motion

Transcutaneous electrical nerve stimulation

400

A 72-year-old man is evaluated for severe left knee pain with swelling that has worsened over the past 5 days. Pain is exacerbated with weight bearing. He has no history of trauma. Current medication is acetaminophen.

On physical examination, temperature is 38.2 °C (100.8 °F). Other vital signs are normal. The left knee is swollen and warm, with erythema, tenderness to palpation, and limited range of motion due to pain. Other joints are normal.

Which of the following is the most appropriate diagnostic test to perform next?

Aspiration of the left knee

Erythrocyte sedimentation rate

Radiography of the left knee

Serum urate level

400

A 57-year-old woman is evaluated for a 2.5-year history of pain and swelling in the hands and wrists. She also has joint stiffness for over an hour after awakening.

On physical examination, vital signs are normal. She has swelling and tenderness of the following: second, third, and fourth proximal interphalangeal joints of the right hand and third and fourth proximal interphalangeal joints of the left hand; the left second and right third metacarpophalangeal joints; and both wrists.

Laboratory evaluation reveals a blood C-reactive protein  level of 4.8 mg/dL (48 mg/L) and positive results on tests for serum rheumatoid factor and anti–cyclic citrullinated peptide antibodies.

Radiographs of the hands show periarticular osteopenia and joint-space narrowing of the proximal interphalangeal joints, periarticular osteopenia and marginal erosions at the second proximal interphalangeal and metacarpophalangeal joints of both hands, and radiocarpal joint-space narrowing.

Which of the following imaging studies should be done next?

Bone scanning

MRI of hands

Ultrasonography of hands

No further imaging

400

A 28-year-old woman is hospitalized at 26 weeks of pregnancy for lower extremity swelling, proteinuria, and elevated blood pressure. Systemic lupus erythematosus was diagnosed last year. Her disease has been well controlled, with stable laboratory values and no recent clinical evidence of disease activity. Her only medication is hydroxychloroquine.

On physical examination, blood pressure is 152/90 mm Hg, and pulse rate is 80/min. Facial swelling, bilateral lower extremity edema, swelling of both hands, and tenderness over the metacarpophalangeal joints are present. She has a gravid abdomen with normal fetal heart tones.

Laboratory studies:


8 Weeks Ago

Current

Platelet count 

Normal

85,000/μL (85 × 109/L)

Alanine aminotransferase

Normal

Normal

Aspartate aminotransferase

Normal

Normal

Serum urate 

4.5. mg/dL (0.30 mmol/L)

Anti–double-stranded DNA antibodies

50

250

C3  complement

85 mg/dL (850 mg/L)

60 mg/dL (600 mg/L)

C4  complement

16 mg/dL (160 mg/L)

4 mg/dL (40 mg/L)

Urinalysis

Normal

3+ protein; no erythrocytes; no leukocytes; no casts

Protein-creatinine ratio 

50 mg/g

320 mg/g

Which of the following is the most appropriate management?

Cyclophosphamide

Immediate delivery

Magnesium sulfate

Prednisone

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