Update on the pathogenesis of Churg-Strauss syndrome

B. Hellmich¹, S. Ehlers², E. Csernok¹, W.L. Gross¹

¹Department of Rheumatology, University Hospital of Schleswig-Holstein, Campus Lübeck, and Rheumaklinik Bad Bramstedt, Lübeck, Germany; ²Division of Molecular Infection Biology, Research Center Borstel, Borstel, Germany.

ABSTRACT
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. 

Please address correspondence to: Bernhard Hellmich, MD, Rheumaklinik Bad Bramstedt, Oskar-Alexander Strasse 26, 24576 Bad Bramstedt, Germany. 
E-mail: hellmich@rheuma-zentrum.de

Clin Exp Rheumatol 2003; 21: (Suppl. 32): S69-S77.
© Copyright Clinical and Experimental Rheumatology 2003.