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Key Features

  • Idiopathic vasculitis of small- and medium-sized arteries seen in patients with symptoms of asthma

  • Affects multiple organ systems, most commonly skin and lung, but heart, gastrointestinal tract, and peripheral nerves may also be affected

Clinical Findings

  • Marked peripheral eosinophilia

  • Chest radiographic findings range from transient opacities to pulmonary nodules

Diagnosis

  • Histologic features include fibrinoid necrotizing epithelioid and eosinophilic granulomas

Treatment

  • Usually requires corticosteroids (prednisone, 1 mg/kg/day, tapering over 3–6 months) and cyclophosphamide (1–2 mg/kg/day, until complete remission is obtained and then tapered slowly)

  • Replace cyclophosphamide with methotrexate or azathioprine for maintenance therapy

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