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

VernacularTitle: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
Author: Fangxing HOU ¹ ; Rongrong HU ; Shuo ZHANG ; Limeng CHEN
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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
Keywords: Glycosylation; Immunoglobulin A; Purpura, Henoch-Sch?nlein; Galactose -deficient IgA1; Henoch-Sch?nlein purpura nephritis; Biomarkers
From: Chinese Journal of Nephrology 2024;40(8):680-684
CountryChina
Language:Chinese
Abstract: Henoch-Sch?nlein purpura is also known as IgA vasculitis. The kidney involvement, namely Henoch-Sch?nlein 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.