中国实用儿科杂志

中国实用儿科杂志 ›› 2025, Vol. 40 ›› Issue (5): 418-424.DOI: 10.19538/j.ek2025050614

• 综述 • 上一篇    下一篇

儿童免疫球蛋白A血管炎发病机制的新认识

  

  1. 浙江大学医学院附属儿童医院肾内科  国家儿童健康临床研究中心， 浙江  杭州  310052
  • 出版日期:2025-05-06 发布日期:2025-08-28
  • 通讯作者: 傅海东，电子信箱：chfhd@zju.edu.cn
  • 基金资助:
    浙江省自然科学基金项目（LTGD24H050002）；浙江省医药卫生技术项目（2024646275）

Update on the pathogenesis of IgA vasculitis in children

  1. Department of Nephrology，The Children’s Hospital，Zhejiang University School of Medicine，National Clinical Research Center for Child Health，Hangzhou  310052，China
  • Online:2025-05-06 Published:2025-08-28

摘要： 免疫球蛋白A血管炎（IgAV）是儿童时期最为普遍的小血管炎症之一。目前，IgAV的确切发病机制尚未完全阐明，但普遍认为其与遗传、免疫系统以及环境因素等多方面因素的相互作用有关。IgAV通常能够自行缓解，然而，部分病例可能发展为肾炎，甚至恶化至终末期肾病。研究显示，不同人群的IgAV发病率存在显著差异，遗传背景在该病的发病过程中扮演了重要角色。半乳糖酶缺乏免疫球蛋白A1（Gd-IgA1）免疫复合物的形成与沉积可能是导致组织损伤的主要原因，尽管其具体作用机制尚存争议。T细胞功能障碍在IgAV的发病过程中具有重要影响。该文探讨遗传因素、免疫功能异常、感染以及内皮细胞损伤等因素在IgAV发病机制中的作用。

关键词: 儿童, 免疫球蛋白A血管炎, 半乳糖酶缺乏免疫球蛋白A1, HLA基因, 免疫功能

Abstract: IgA vasculitis（IgAV），is one of the most common forms of small blood vessel inflammation in children. While the precise pathogenesis of IgAV remains to be fully understood，it is widely accepted that the condition involves a complex interaction of genetics，immune system，and environmental factors.Typically，IgAV resolves on its own；however，in some cases，it can progress to nephritis or even end-stage renal disease.Researches indicate that there are significant variations in the incidence of IgAV across different populations，with genetic predisposition playing a crucial role in the development of the disease.Furthermore，studies suggest that the formation and deposition of galactose-deficient（Gd-）IgA1 immune complexes may be the primary cause of tissue damage，although the exact mechanism is still a matter of controversy.T cell dysfunction has a significant effect on the pathogenesis of IgAV.In this review，we will study the roles of genetic factors，immune dysfunction，infections，and endothelial cell injury in the pathogenesis of IgA vasculitis.

Key words: child, IgA vasculitis, galactose-deficient IgA1, HLA gene, immune function