成人发病型 IgA 血管炎相关性肾炎导航

Life Pub Date : 2024-07-25 DOI:10.3390/life14080930
Ming Ying Gan, Freda Zhi Yun Chua, Z. Y. Chang, Yan Ting Chua, G. Chan
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摘要

审查目的:IgA 血管炎(IgAV),前身为过敏性紫癜(Henoch-Schonlein purpura),是儿童时期最常见的全身性血管炎。然而,人们对成人的这种疾病知之甚少,而且这种疾病的病情更严重,预后更差。因此有必要进行早期诊断和治疗。综述范围:我们描述了成人 IgAV 的病理生理学、临床表现和诊断。IgAV-IgA血管炎相关性肾炎(IgAVN)中肾小球肾炎的发病率较高,这通常是导致不良预后的原因。因此,我们还旨在总结有关 IgAVN 治疗策略的最新临床数据。IgAVN和IgA肾病(IgAN)的诊断和组织学鉴别仍然是一项挑战。回顾治疗疗法:IgAVN 和 IgAN 的病理机制似乎是一致的,IgAN 的数据经常被推断到 IgAVN 上。在本综述中,我们将讨论免疫抑制疗法的使用,并重点介绍有关 IgAVN/IgAN 新出现和有前景的新型疗法的证据。我们撰写这篇综述的目的在于指导未来的治疗策略和未来的研究方向。
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Navigating Adult-Onset IgA Vasculitis-Associated Nephritis
Purpose of Review: IgA vasculitis (IgAV), formerly Henoch–Schonlein purpura, is the most common systemic vasculitis in childhood. In adults, however, this condition is poorly understood, yet associated with more severe disease and poorer outcomes. This necessitates the need for early diagnosis and management. Scope of Review: We describe the pathophysiology, clinical manifestations, and diagnosis of IgAV in adults. Poor outcomes are often due to the high frequency of glomerulonephritis in IgAV-IgA vasculitis-associated nephritis (IgAVN). We hence also aim to summarize the latest clinical data regarding treatment strategies in IgAVN. The diagnosis and differentiation in histology between IgAVN and IgA nephropathy (IgAN) remain a challenge. Review of treatment therapies: Pathological mechanisms between IgAVN and IgAN appear to be consistent between the two, and data from IgAN are often extrapolated to IgAVN. The role of various immunosuppression therapies remains controversial, and in this review, we will discuss immunosuppression use and highlight evidence surrounding emerging and promising novel therapies in IgAVN/IgAN. Our aim for this review is to guide future treatment strategies and direct future studies.
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