IgA肾病发病机制的研究进展  

Researches progress on pathogenesis of IgA nephropathy

作　　者：王淦淦 郑可 李雪梅 Wang Gangan;Zheng Ke;Li Xuemei(Chinese Academy of Medical Sciences,Peking Union Medical College,Department of Nephrology,Peking Union Medical College Hospital,Beijing 100730,China)

机构地区：[1]中国医学科学院、北京协和医学院、北京协和医院肾内科,北京100730

出　　处：《中华肾脏病杂志》2024年第3期225-230,共6页Chinese Journal of Nephrology

基　　金：北京协和医学院教学改革项目(2021zlgc0125);北京市科技计划(D181100000118001);中央高水平医院临床科研专项(2022-PUMCH-B-021)。

摘　　要：IgA肾病(IgA nephropathy,IgAN)是目前全球范围内最常见的原发性肾小球肾炎,20%~40%的患者在诊断后20年内进展为终末期肾病。IgAN的发病机制尚不明确,临床治疗主要以控制病情进展为主,缺乏特异性治疗方案。围绕半乳糖缺乏的IgA1(galactose-deficient IgA1,Gd-IgA1)开展的一系列研究提示IgAN发病涉及多个环节。该文从IgA1的结构特征、IgAN患者体内Gd-IgA1抗体及含Gd-IgA1免疫复合物、Gd-IgA1免疫复合物在肾脏中的沉积、Gd-IgA1免疫复合物沉积对肾脏的损伤、补体在IgAN中的作用、IgA肾病的基因组学及黏膜免疫与IgAN等方面,综述了IgAN发病机制的研究进展,为进一步的研究和临床治疗提供线索和思路。IgA nephropathy(IgAN)is currently the most common primary glomerulonephritis worldwide,with 20%-40%of patients progressing to end-stage renal disease within 20 years of diagnosis.At present,the pathogenesis of IgAN is not clear,and clinical treatment is mainly to control the progression,without specific treatment plan.A series of studies on galactose-deficient IgA1(Gd-IgA1)suggest that the pathogenesis of IgAN involves multiple links.This review summarizes the research progress on the pathogenesis of IgAN,covering the structure characteristics of IgA1,Gd-IgA1 antibodies and Gd-IgA1 immune complexes in IgAN patients,the deposition of Gd-IgA1 immune complexes in the kidneys,kidney damage following the deposition of Gd-IgA1 immune complexes,the role of complement in IgAN,the genomics of IgAN,and mucosal immunity in IgAN,providing clues and insights for further research and clinical treatment.

关 键 词：肾小球肾炎 IgA 免疫球蛋白A 抗原抗体复合体 半乳糖缺乏的IgA1 多重打击假说 发病机制 

分 类 号：R692.31[医药卫生—泌尿科学]