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.
| Original language | English |
|---|---|
| Journal | Clinical and Experimental Rheumatology |
| Volume | 21 |
| Issue number | 6 SUPPL. 32 |
| Pages (from-to) | 69-77 |
| Number of pages | 9 |
| ISSN | 0392-856X |
| Publication status | Published - 2003 |
Research Areas and Centers
- Academic Focus: Center for Infection and Inflammation Research (ZIEL)
