免疫球蛋白 A 血管炎:临床特征和病理生理学。

The Kaohsiung journal of medical sciences Pub Date : 2024-07-01 Epub Date: 2024-06-03 DOI:10.1002/kjm2.12852
Ya-Chiao Hu, Yao-Hsu Yang, Bor-Luen Chiang
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引用次数: 0

摘要

可触及的紫癜、胃肠道症状、关节受累和肾脏疾病是免疫球蛋白 A 血管炎(IgAV)的特征。从轻度蛋白尿到重度肾炎或肾病综合征的肾脏受累,突出了监测 IgAV 患者肾功能的重要性。识别这些关键特征对于早期诊断和适当治疗以预防与肾病相关的长期并发症至关重要。然而,IgAV 的发病机制仍不清楚。疾病机制涉及多种因素,包括异常糖基化的 IgA、抗内皮细胞抗体和中性粒细胞感染诱因的相互作用,这些是 IgAV 的主要致病机制。2019 年与冠状病毒疾病相关的 IgAV 病例为我们进一步了解感染与 IgAV 发病机制之间的联系提供了新的视角。这篇综述为医疗保健专业人员和风湿病学研究人员提供了宝贵的资源,帮助他们更好地了解 IgAV 的临床特征和病理生理学。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Immunoglobulin A vasculitis: The clinical features and pathophysiology.

Palpable purpura, gastrointestinal symptoms, joint involvement, and renal disease characterize immunoglobulin A vasculitis (IgAV). Renal involvement ranging from mild proteinuria to severe nephritic or nephrotic syndrome highlights the importance of monitoring kidney function in patients with IgAV. Recognizing these key features is crucial for early diagnosis and appropriate management to prevent long-term complications related to kidney disease. However, the pathogenesis of IgAV remains unclear. Disease mechanisms involve various factors, including the interplay of aberrantly glycosylated IgA, anti-endothelial cell antibodies, and neutrophils following infection triggers, which are the main pathogenic mechanisms of IgAV. Insights from cases of IgAV related to Coronavirus disease 2019 have offered additional understanding of the connection between infection and IgAV pathogenesis. This review provides a valuable resource for healthcare professionals and rheumatology researchers seeking a better understanding of the clinical features and pathophysiology of IgAV.

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