IgA 肾病:新出现的疾病机理。

Pub Date : 2024-07-01 Epub Date: 2024-06-24 DOI:10.25259/ijn_425_23
Lydia E Roberts, Chloe E C Williams, Louise Oni, Jonathan Barratt, Haresh Selvaskandan
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引用次数: 0

摘要

免疫球蛋白 A 肾病(IgAN)是全球报告的最常见的原发性肾小球肾炎,其特点是免疫球蛋白 A(IgA)系膜沉积,O-糖基化不良。这种沉积会导致一连串的肾小球和肾小管间质炎症和纤维化,进而发展为慢性肾病。进展速度的变化反映了导致 IgAN 的多种遗传和环境因素。在此,我们总结了当代对驱动 IgAN 的疾病机制的理解,并概述了针对这些机制的新兴疗法。
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IgA Nephropathy: Emerging Mechanisms of Disease.

Immunoglobulin A nephropathy (IgAN) is the most common primary glomerulonephritis reported across the world and is characterized by immunoglobulin A (IgA) dominant mesangial deposits, which are poorly O-glycosylated. This deposition leads to a cascade of glomerular and tubulointerstitial inflammation and fibrosis, which can progress to chronic kidney disease. The variability in rate of progression reflects the many genetic and environmental factors that drive IgAN. Here, we summarize the contemporary understanding of the disease mechanisms that drive IgAN and provide an overview of new and emerging therapies, which target these mechanisms.

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