[Churg-Strauss syndrome]

Abstract

Churg-Strauss syndrome (CSS) is a rare systemic vasculitis occurring in patients with a history of asthma or allergy and eosinophilia. Although lungs, skin, and peripheral nervous system are the most common sites of involvement, many other organs, including heart, kidneys, and gastrointestinal tract, can be affected. CSS is an eosinophil-mediated disease, but its pathophysiology is not fully known. There are no specific laboratory tests for CSS. The main laboratory characteristics are peripheral blood eosinophilia, elevated serum IgE levels, and the presence of an inflammatory syndrome. Antineutrophil cytoplasmic antibodies directed against myeloperoxidase are positive in approximately one-half of patients. The diagnosis of CSS rests on the association of the clinical features of the disease with its histologic hallmarks. The biopsy of affected tissues may show a spectrum of histologic changes, including leukocytoclastic or necrotizing vasculitis, eosinophilic tissue infiltration, vascular and extravascular granulomas. The involved vessels are small arteries, veins, arterioles, venules, and capillaries. Corticosteroids, sometimes in combination with a cytotoxic agent, such as cyclophosphamide, are the most effective treatment of CSS. Intravenous immunoglobulins or interferon-alpha may be useful for patients refractory to conventional treatment.