Rheumatology
Prepared by Christopher Allen, MD, Resident, Department of Medicine, and Gregory Chipman, MD, Chief Resident, Case Western Reserve University School of Medicine, Louis Stokes VA Medical Center, Cleveland, Ohio
A 36-year-old man presented with 2 weeks of progressive lower abdominal pain associated with nausea, vomiting, and 2 bloody bowel movements. Physical examination revealed a tender abdomen, with normoactive bowel sounds and erythematous plaques on the extensor surfaces of the elbows, knees (Figure 1), and metacarpophalangeal joints. Hyperreflexia was also evident. Upon his return from the first Gulf War, he was diagnosed with asthma. Skin plaque biopsy revealed a focal perivascular infiltrate of histiocytes and eosinophils, and a colonoscopy with random biopsies showed acute and chronic inflammation with eosinophilia (Figure 2). An echocardiogram revealed a left ventricular ejection fraction of 30%. Laboratory test results revealed 38% eosinophils and immunoglobulin E level of 844 IU. A diagnostic biopsy of the sural nerve demonstrated necrotizing vasculitis and eosinophilia involving the small muscular arteries (Figure 3).
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| Figure 1 |
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| Figure 2 |
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| Figure 3 |
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What's Your Diagnosis?
- Wegener's granulomatosis
- Polyarteritis nodosa
- Churg-Strauss syndrome
- Microscopic polyangiitis
Quiz Answer
Churg-Strauss syndrome—This patient exhibits many of the typical features of Churg-Strauss syndrome. This condition may involve any organ, but involvement of the lungs, skin, cardiovascular system, kidneys, peripheral nervous system, or the gastrointestinal (GI) tract is common. Patients often present with nonspecific complaints of fever, malaise, anorexia, and weight loss characteristic of a multisystem disease.1 Pulmonary findings usually dominate the clinical picture, with severe asthma attacks. Clinical signs of heart disease are common, as are cutaneous lesions; the latter include purpura and cutaneous and subcutaneous nodules. Peripheral neuropathy is seen in up to 75% of cases, often as a mononeuritis multiplex. Abdominal pain is seen in close to 60% of patients as an eosinophilic gastroenteritis.1
Also known as allergic granulomatosis and angiitis, Churg-Strauss syndrome is classified as a vasculitis involving the small- and medium-sized muscular arteries, capillaries, veins, and venules. Histopathologic features include necrotizing vasculitis, infiltration by eosinophils (Figure 4), and extravascular granulomas. Surgical lung biopsy had long been considered the gold standard for diagnosis. However, the American College of Rheumatology recently established the following 6 criteria for the diagnosis of Churg-Strauss. The presence of 4 or more of these findings yields a sensitivity of 85% and a specificity of 99.7% for Churg-Strauss syndrome2:
- Asthma
- Eosinophilia, >10%
- Neuropathy, mono/poly
- Pulmonary infiltrates, nonfixed
- Paranasal sinus abnormality
- Extravascular eosinophils
The prognosis is poor without treatment, but treatment provides substantial increase in the survival rate. Azotemia, proteinuria, GI tract involvement, cardiomyopathy, and central nervous system involvement contribute to poor outcomes. Most deaths are seen as a result of cardiac failure, cerebral hemorrhage, renal failure, GI bleeding, or asthma. High-dose corticosteroid therapy is the mainstay of treatment.
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| Figure 4 |
Wegener's granulomatosis typically produces granulomatous inflammation of the upper and lower respiratory tract and necrotizing glomerulonephritis of the kidneys. Microscopic polyangiitis is similar to Churg-Strauss syndrome, but without granulomatous formation. Polyarteritis nodosa can involve nearly any organ system but typically spares the lungs. This clinical presentation of multisystem involvement, along with peripheral and tissue eosinophilia, distinguishes Churg-Strauss syndrome from the other vasculitides.
References
- Noth I, Strek ME, Leff AR. Churg-Strauss syndrome. Lancet. 2003; 361:587-594.
- Masi AT, Hunder GG, Lie JT, et al. The American College of Rheumatology 1990 criteria for the classification of Churg-Strauss syndrome (allergic granulomatosis and angiitis). Arthritis Rheum. 1990; 33:1094-1100.
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