中国实用儿科杂志

中国实用儿科杂志 ›› 2022, Vol. 37 ›› Issue (1): 12-16.DOI: 10.19538/j.ek2022010603

• 专题笔谈 • 上一篇    下一篇

从过敏性紫癜到IgA血管炎——发病机制新认识

  

  1. 南京大学医学院附属金陵医院（东部战区总医院）  南方医科大学南京临床医学院（东部战区总医院）  南京医科大学附属金陵临床医学院（东部战区总医院）儿科，江苏  南京  210002
  • 出版日期:2022-01-06 发布日期:2022-02-18
  • 通讯作者: 高春林，电子信箱：shuangmu34@163.com

From Henoch-Schönlein purpura to IgA vasculitis: A new insight of pathogenesis

  1. Department of Pediatrics，Jinling Hospital，the First School of Clinical Medicine，Southern Medical University，Nanjing  210002，China
  • Online:2022-01-06 Published:2022-02-18

摘要： 过敏性紫癜为儿童期最常见的系统性小血管炎，早期被认为是过敏性疾病，随着免疫病理技术的发展，将其定义为IgA血管炎，累及肾脏的称为IgA血管炎肾炎（IgAVN）或紫癜性肾炎。其发病机制存在两种假说，其一为类似于IgA肾病（IgAN）的四重打击学说，其二为围绕血管炎症和黏膜免疫为主的新多重打击学说，认为IgAVN是一种双重疾病，兼具有IgAN的Gd-IgA1异常和IgAV的血管炎发生机制。文章就其进展进行以下综述。

关键词: 过敏性紫癜, IgA血管炎, 紫癜性肾炎, 发病机制 

Abstract: Henoch-Schönlein purpura is the most common systemic vasculitis in childhood，which was considered as an allergic disease in the early stage. With the development of immunopathology technique，it is now defined as IgA vasculitis，and when involving the kidney it is called IgA vasculitis nephritis（IgAVN） or Henoch-Schönlein purpura nephritis. There are two hypotheses concerning the pathogenesis of IgAV（N），one is the four-hit hypothesis similar to IgA nephropathy（IgAN），and the other is the novel multi-hit hypothesis centered on vascular inflammation and mucosal immunity. The latter hypothesis holds that IgAVN is a dual disease，which has the Gd-IgA1 abnormalities of IgAN and the vasculitis pathogenesis of IgAV. Here we review the up-to-date studies.

Key words: Henoch-Sch?nlein purpura, IgA vasculitis, Henoch-Sch?nlein purpura nephritis, pathogenesis