Moosig F, Hellmich B
Klinik für Rheumatologie und Immunologie, Klinikum Bad Bramstedt GmbH, Universitätsklinikum Schleswig Holstein, Oskar-Alexander-Str. 26, 24576, Bad Bramstedt, Deutschland.
Z Rheumatol. 2012 Nov;71(9):765-70. doi: 10.1007/s00393-012-0985-9.
The Churg-Strauss syndrome (CSS) is the rarest subtype of the so-called anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV) and has the lowest frequency of ANCA-positivity (around 30%). In addition to asthma and blood eosinophilia, CSS is characterized by end-organ damage, which can be caused by either vasculitis and/or tissue infiltration of eosinophilic granulocytes. The CSS shares many etiological and clinical features of other hypereosinophilic syndromes. Recently, a distinct genetic background could be demonstrated for both the ANCA-positive and ANCA-negative subtypes of CSS as compared to the other two forms of AAV. Among other cytokines, interleukin-5 (IL-5) could be identified as a key mediator of eosinophilia. Therefore, recent clinical trials in CSS aimed to target IL-5. Outside of clinical trials, treatment of CSS is adapted to disease stage and activity, as recommended for other types of AAV.
变应性肉芽肿性血管炎（CSS）是所谓抗中性粒细胞胞浆抗体（ANCA）相关性血管炎（AAV）中最罕见的亚型，ANCA阳性率最低（约30%）。除哮喘和血液嗜酸性粒细胞增多外，CSS的特征还包括终末器官损伤，这可能由血管炎和/或嗜酸性粒细胞的组织浸润引起。CSS与其他嗜酸性粒细胞增多综合征有许多病因和临床特征。最近发现，与其他两种形式的AAV相比，CSS的ANCA阳性和ANCA阴性亚型都有独特的遗传背景。在其他细胞因子中，白细胞介素-5（IL-5）被确定为嗜酸性粒细胞增多的关键介质。因此，最近针对CSS的临床试验旨在靶向IL-5。在临床试验之外，CSS的治疗根据疾病阶段和活动情况进行调整，这与其他类型的AAV的治疗建议相同。
