张金玉1,,
曹玉涵1,,,
叶寅寅1,
汪裕伟1,
杨沿浪1,
杨慧2
1. 皖南医学院附属弋矶山医院 肾脏内科, 安徽 芜湖 241001
2. 复旦大学 医学院 免疫学系, 上海 200032
基金项目: 国家自然科学基金青年基金项目(31900974;81702092)
详细信息 作者简介: 张金玉(1989-), 女, 住院医师。E-mail: zhangjinyu9152@163.com
通讯作者: 曹玉涵, 副教授。E-mail: cyh880130@163.com 中图分类号: R593.2
摘要:抗中性粒细胞胞浆抗体(anti-neutrophil cytoplasmic antibody,ANCA)相关性血管炎(ANCA-associated vasculitis,AAV)是一组影响中小血管的、严重危及生命的系统性自身免疫性疾病。AAV的病因和发病机制是多因素的,最初人们并不认为补体系统与AAV的发展有关。然而,动物及临床研究表明补体系统的激活,特别是旁路途径,对AAV的发展至关重要,补体激活产物C5a具有核心作用。本文主要对补体系统在AAV发病机制中的研究进展进行综述。
关键词: 抗中性粒细胞胞浆抗体/
血管炎/
补体
Abstract:Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a group of life-threatening systemic autoimmune diseases that affect small and medium vessels. The etiology and pathogenesis of AAV are multifactorial, and initially the complement system was not thought to be associated with the development of AAV. Evidence from animal models and clinical observations indicate, however, that activation of the complement system, especially the alternative pathway is crucial for the development of AAV, and the complement activation product C5a has a central role.The paper mainly summarizes the mechanism of complement system activation in the pathogenesis of AAV.
Keywords:anti-neutrophil cytoplasmic antibody/
vasculitis/
complement
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