Churg–Strauss syndrome is a small-vessel necrotizing vasculitis typically characterized by asthma, lung infiltrates, extravascular necrotizing granulomas and hypereosinophilia. The most recent clinical studies on its pathogenesis and therapeutic management are reviewed here.
French and Italian clinical studies found that the clinical characteristics of patients with Churg–Strauss syndrome differed according to their antineutrophil cytoplasmic autoantibody status: cardiomyopathy predominated in antineutrophil cytoplasmic autoantibody-negative patients while necrotizing glomerulonephritis was more often observed in antineutrophil cytoplasmic autoantibody-positive patients. These histologically documented findings suggest the existence of different Churg–Strauss syndrome subtypes, characterized by the predominance of distinct pathogenetic mechanisms. To date, following the therapeutic recommendations for Churg–Strauss syndrome (i.e. corticosteroids and, when required, immunosuppressants), patient outcomes are good, with 5-year survival exceeding 90%, but often with the need to continue low-dose corticosteroids to control residual asthma.
The precise pathogenetic mechanisms of Churg–Strauss syndrome are only partly elucidated. Recent results suggest that antineutrophil cytoplasmic autoantibodies are probably more involved in the vasculitic manifestations of Churg–Strauss syndrome (e.g. glomerulonephritis) whereas eosinophil tissue infiltration and associated cytotoxicity would be responsible for cardiomyopathy. If confirmed, these results could support individual therapeutic stratification according to the clinical pattern. Furthermore, some patients may benefit from new biologic therapies under development, for example antiinterleukin-5 or antiimmunoglobulin E monoclonal antibodies.
Department of Internal Medicine, French Vasculitis Study Group, Centre de Référence Vascularites et Sclérodermies, Hôpital Cochin, Assistance Publique–Hôpitaux de Paris, Université Paris–Descartes, Paris, France
Correspondence to Dr Christian Pagnoux, Department of Internal Medicine, Hôpital Cochin, 27, rue du Faubourg Saint-Jacques, 75679 Paris Cedex 14, France Tel: +33 1 58 41 14 61; fax: +33 1 58 41 14 60; e-mail: firstname.lastname@example.org