Update on the pathogenesis of Churg-Strauss syndrome.
Churg-Strauss syndrome (CSS) is a rare form of systemic vasculitis occurring in patients with asthma. The cause of CSS is unknown, and yet little data are available regarding its pathogenesis. The presence of a marked tissue- and blood-eosinophilia, as well as secretory products of eosinophils in blood and tissues, implicates a pathogenetic role of eosinophil granulocytes. Prolonged survival of eosinophils due to inhibition of CD95-mediated apoptosis by soluble CD95 seems to contribute to eosinophilia in CSS. Although the mechanisms involved in eosinophil-activation in CSS have not been elucidated, recent data suggest a possible role of T lymphocytes secreting eosinophil-activating cytokines. This review describes the current insights into the pathogenesis of CSS in the light of its putative nature as a type 2 granulomatous disease. Recent clinical, experimental and epidemiologic data regarding the possible role of inflammatory cells and their secretory products, anti neutrophil cytoplasm antibodies (ANCA), epidemiologic factors and anti-asthma treatments are summarized.
Hellmich
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Department of Rheumatology, University Hospital of Schleswig-Holstein, Campus Lübeck, Germany. hellmich@rheuma-zentrum.de
Ehlers
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Csernok
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Gross
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D015267:Churg-Strauss Syndrome / Q000209:etiology* / Q000276:immunology* / Q000473:pathology; D004804:Eosinophils / Q000276:immunology*; D006801:Humans