Eosinophilic Granulomatosis with Polyangiitis (formerly Churg-Strauss Syndrome)

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