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Self-Assessment Questions


Systemic Vasculitis: Review Questions

Alexandru F. Kimel, MD,
and Neil J. Gonter, MD

Dr. Kimel is a rheumatology fellow, University of Medicine and Dentistry of New Jersey-Robert Wood Johnson School of Medicine, New Brunswick, NJ.
Dr. Gonter is an assistant clinical professor of medicine, Columbia University, New York, NY, and a practicing rheumatologist, Rheumatology Associates of North Jersey, Teaneck, NJ.

Choose the single best answer for each question.

Questions 1 to 3 refer to the following case.
A 74-year-old man with a history of benign prostatic hyperplasia, hypertension, type 2 diabetes mellitus, and glaucoma presents to the emergency department with a 2-week history of paroxysmal fevers up to 101°F, decreased appetite with sudden neck and tongue pain when chewing food, and new-onset progressive blurry vision in his right eye. Laboratory testing reveals a hemoglobin A1c (HbA1c) of 5.5%, hemoglobin level of 14 g/dL, prostate-specific antigen level of 2.1 ng/mL, and an erythrocyte sedimentation rate (ESR) greater than 115 mm/hr. Blood and urine cultures are negative. Urinalysis is unremarkable.

1. What is the most appropriate action at this time?

  1. Administer intravenous (IV) antibiotics and antifungal medications
  2. Administer IV methylprednisolone 1000 mg
  3. Call ophthalmology to perform an urgent tonometric study
  4. Repeat blood cultures with the next fever spike
  5. Start prednisone 20 mg daily
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2. Which of the following should be performed to establish the diagnosis?

  1. Computed tomography (CT) angiogram of the thorax
  2. Cranial ultrasound
  3. 2-Dimensional Doppler echocardiogram
  4. Fundoscopic examination
  5. Temporal artery biopsy
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3. How should this patient be treated?

  1. IV infliximab every 8 weeks
  2. Methotrexate 25 mg/wk with folic acid 1 mg/day
  3. Prednisone 60 mg/day
  4. Prednisone 60 mg/day and aspirin 81 mg/day
  5. Timolol 0.5% gtt twice daily
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Questions 4 and 5 refer to the following case.
A 38-year-old man with newly diagnosed hypertension presents to his primary care physician with multiple lesions on his lower extremities, a 10-lb weight loss over the past 4 months, and dull discomfort of his right testicle. The lesions started out as dark hyperpigmented areas, which ulcerated and healed, leaving a crater-like scab (Figure 1). The lesions are not pruritic or painful. He has been seen by a wound care specialist, but the recommended treatments have not helped. Cultures taken on several occasions have been unremarkable. The patient has recently developed some new dark spots on the tips of his fingers and toes, with increasing tingling and numbness in these areas. On physical examination, blood pressure is 160/94 mm Hg, heart rate is 88 bpm, respiratory rate is 14 breaths/min, and temperature is 100.5°F. Laboratory results reveal a hemoglobin level of 15.5 g/dL, platelet count of 247,000 cells/µL, blood urea nitrogen (BUN) of 47 mg/dL, serum creatinine of 1.8 mg/dL, aspartate aminotransferase of 25 U/L, alanine aminotransferase of 29 U/L, alkaline phosphatase of 220 U/L, and ESR of 88 mm/hr. Antineutrophil cytoplasmic antibody (ANCA) staining is negative, and hepatitis A and C serologies are negative. Serologic testing for hepatitis B surface antigen is positive, and testing for HIV is negative. The patient drinks alcohol occasionally and does not smoke.

Figure 1

4. This patient’s symptoms are most consistent with which of the following conditions?

  1. Churg-Strauss syndrome (CSS)
  2. Henoch-Schönlein purpura
  3. Polyarteritis nodosa (PAN)
  4. Thromboangiitis obliterans
  5. Wegener’s granulomatosis
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5. Which of the following should be performed to confirm this patient’s diagnosis?

  1. Kidney biopsy
  2. Muscle biopsy
  3. Peripheral nerve biopsy
  4. Renal angiogram
  5. Testicular ultrasound
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6. A 42-year-old woman presents to her physician with shortness of breath, fatigue, mild fevers, and a 12-lb weight loss over the past 8 weeks. She has had asthma since she was a teenager, with mild attacks occurring every 2 to 3 months, usually alleviated with an albuterol inhaler. She notes an ongoing dry cough during these attacks. The patient reports lost sensation in her right arm approximately 2 weeks ago as well as in her left arm approximately 2 months ago. She has had atopic dermatitis and eczema on several occasions. Laboratory testing reveals a hemoglobin level of 14 g/dL; white blood cell count of 98,000 cells/µL, with a differential of 28% neutrophils, 7% lymphocytes, 24% eosinophils, and 1% basophils; BUN of 12 mg/dL; and serum creatinine level of 1.4 mg/dL. The ESR is 65 mm/hr, and C-reactive protein is 3.2 mg/dL. P-ANCA staining is positive with confirmed myeloperoxidase reactivity. Urinalysis shows numerous white and red blood cells. Urine cultures are negative. Chest radiograph demonstrates bilateral diffuse interstitial infiltrates. This patient’s symptoms are consistent with which of the following?

  1. Allergic bronchopulmonary aspergillosis
  2. CSS
  3. Eosinophilia-myalgia syndrome
  4. Pulmonary sarcoidosis
Click here to compare your answer.

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