Eosinophilic Granulomatosis with Polyangiitis (Churg–Strauss Syndrome)

Abstract

Eosinophilic granulomatosis with polyangiitis (EGPA; Churg–Strauss syndrome) is a rare, systemic, necrotizing, small-sized vessel vasculitis. It most typically affects middle-aged individuals who have developed late-onset asthma then more characteristic vasculitis manifestations, like fever, skin lesions (40–75 % of the patients, mainly purpura, often necrotic, and/or cutaneous nodules or papules and sometimes migratory urticarial rashes) and/or mononeuritis multiplex. Blood and tissue eosinophilia, especially the lungs, gastrointestinal tract, and heart, is a hallmark of the disease. Biopsy of an affected organ or lesions, especially skin lesions, can help in making the diagnosis, when showing eosinophilic and/or granulomatous vasculitis, with or without necrosis. Antineutrophil cytoplasm autoantibodies (ANCA) can be detected in nearly 40 % of the patients. Differential diagnoses include drug-induced and hypersensitivity vasculitis, parasitic infections, and hypereosinophilic syndromes. Treatment relies on systemic corticosteroids, combined with an immunosuppressant for severe and/or life-threatening forms.