异常糖基化IgA1在紫癜性肾炎发病机制和临床应用中的研究进展  

Research progress on the pathogenesis and clinical application of abnormal glycosylation of IgA1 in Henoch-Schonlein purpura nephritis

作　　者：侯方兴 胡蓉蓉 张硕 陈丽萌 Hou Fangxing;Hu Rongrong;Zhang Shuo;Chen Limeng(Department of Nephrology,State Key Laboratory of Complex Severe and Rare Diseases,Peking Union Medical College Hospital,Chinese Academy of Medical Science and Peking Union Medical College,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、疑难重症及罕见病国家重点实验室(北京协和医院),北京100730

出　　处：《中华肾脏病杂志》2024年第8期680-684,共5页Chinese Journal of Nephrology

基　　金：新一代人工智能国家科技重大专项(2022ZD0116003);国家自然科学基金(82170709);北京市示范性研究型病房建设项目(BCRW202001);中国医学科学院医学与健康科技创新工程项目(CIFMS 2021-I2M-1-003);中央高水平医院临床科研业务费(2022-PUMCH-B-019、2022-PUMCH-D-002)。

摘　　要：过敏性紫癜又称为IgA血管炎,肾脏受累即紫癜性肾炎(Henoch-Schonlein purpura nephritis,HSPN)是其主要临床表现之一。半乳糖缺陷型IgA1在HSPN发病中发挥了重要作用,其机制与IgA肾病“四重打击假说”相似,IgA1在肾小球系膜区沉积致病,还与炎性反应密切相关,且其致病免疫复合物组成更复杂。因此,异常糖基化IgA1有望成为诊断和预测HSPN发病、判断疾病活动性和预测预后的生物标志物。该文简述了近年来异常糖基化IgA1在HSPN发病机制及临床应用中的研究进展。Henoch-Schonlein purpura is also known as IgA vasculitis.The kidney involvement,namely Henoch-Schonlein purpura nephritis(HSPN),is one of IgA vasculitis's main clinical manifestations.Galactose-deficient IgA1 plays an important role in the pathogenesis of HSPN,which not only is similar to the"four-hits hypothesis"of IgA nephropathy,leading to IgA1 deposit in the mesangial region of the kidney,but also is closely related to inflammation with more complicated compositions of its immune complex.Therefore,abnormal glycosylation of IgA1 is expected to be a biomarker for diagnosis and prediction of HSPN pathogenesis,disease activity determination and prognosis prediction.Here,the paper reviews the research progress on the pathogenesis and clinical application of abnormal glycosylation of IgA1 in HSPN.

关 键 词：糖基化 免疫球蛋白A 紫癜 过敏性 半乳糖缺陷型IgA1 紫癜性肾炎 生物标志物 

分 类 号：R692.34[医药卫生—泌尿科学]