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中国临床药理学与治疗学 ›› 2018, Vol. 23 ›› Issue (2): 235-240.doi: 10.12092/j.issn.1009-2501.2018.02.022

• 综述与讲座 • 上一篇    

抗中性粒细胞胞质抗体相关性小血管炎的机制与治疗进展

余淑媛1,郑 麟1,韩 飞2   

  1. 1 浙江江山市人民医院风湿免疫科,江山 324100,浙江; 2 浙江大学医学院附属第一医院肾脏病中心浙江省肾脏病防治技术研究重点实验室,国家中医药管理局三级实验室“肾脏免疫实验室”,杭州 310003,浙江
  • 收稿日期:2017-07-31 修回日期:2017-08-29 出版日期:2018-02-26 发布日期:2018-03-02
  • 作者简介:余淑媛,女,本科,主治医师,主要从事肾内科临床工作。 Tel: 13587015156 E-mail: 6684867@qq.com

Pathogenesis and treatment progress of antineutrophil cytoplasmic antibody-associated vasculitis

YU Shuyuan1, ZHENG Lin1, HAN Fei2   

  1. 1 Department of Nephrology, Jiangshan People's Hospital,Jiangshan 324100, Zhejiang, China; 2 Kidney Disease Center, the First Affiliated Hospital, College of Medicine, Zhejiang University, Hangzhou 310003, Zhejiang, China
  • Received:2017-07-31 Revised:2017-08-29 Online:2018-02-26 Published:2018-03-02

摘要:

血管炎是一类可引起血管非特异性炎症和坏死的自身免疫性疾病。按血管受累的大小分类,包括了小血管炎、中等血管炎及大血管炎。抗中性粒细胞胞质抗体(ANCA)相关性小血管炎(AAV)属于小血管炎的范畴,主要包含了肉芽肿性多血管炎(GPA),显微镜下多血管炎(MPA)和嗜酸肉芽肿性多血管炎(EGPA)。它们可累及全身不同脏器,活动期异常凶险,治疗不及时可致高死亡率。本文简要综述AAV的发病机制及治疗进展。

关键词: 抗中性粒细胞胞质抗体相关性小血管炎, 环磷酰胺, 利妥昔单抗

Abstract:

Vasculitis is an autoimmune disease which may cause nonspecific inflammation and necrosis of the blood vessels. According to degree of the vascular involvement, vasculitis divides into small vasculitis, moderate vasculitis and large vasculitis. Antineutrophil cytoplasmic antibody (ANCA) associated vasculitis (AAV) which belongs to the small vasculitis includes granulomatous polyangiitis (GPA) microscopic polyangiitis (MPA) and eosinophilic granulomatous polyangiitis (EGPA). Different organs of the whole body could be involved and the active period is extremely dangerous. Untimely treatment can result in high mortality. This paper briefly reviews the pathogenesis and treatment progress of AAV.

Key words: vasculitis antineutrophil cytoplasmic antibody-associated (AAV), cyclophosphamide (CTX), rituximab (RTX)

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