Hellmich B, Ehlers S, Csernok E, Gross W L
Department of Rheumatology, University Hospital of Schleswig-Holstein, Campus Lübeck, Germany.
Clin Exp Rheumatol. 2003 Nov-Dec;21(6 Suppl 32):S69-77.
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.
变应性肉芽肿性血管炎（CSS）是一种发生于哮喘患者的罕见的系统性血管炎。CSS的病因不明，关于其发病机制的数据也很少。显著的组织和血液嗜酸性粒细胞增多，以及血液和组织中嗜酸性粒细胞的分泌产物，提示嗜酸性粒细胞在发病机制中起作用。可溶性CD95抑制CD95介导的细胞凋亡导致嗜酸性粒细胞存活时间延长，这似乎是CSS嗜酸性粒细胞增多的原因。虽然CSS中嗜酸性粒细胞激活的机制尚未阐明，但最近的数据表明分泌嗜酸性粒细胞激活细胞因子的T淋巴细胞可能起作用。这篇综述根据CSS作为2型肉芽肿性疾病的假定性质，描述了目前对其发病机制的见解。总结了关于炎症细胞及其分泌产物、抗中性粒细胞胞浆抗体（ANCA）、流行病学因素和抗哮喘治疗可能作用的近期临床、实验和流行病学数据。
