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肾脏病学中的补体系统抑制剂:最新叙述性综述

Complement System Inhibitors in Nephrology: An Update-Narrative Review.

作者信息

Apetrii Mugurel, Costache Alexandru Dan, Costache Enache Irina Iuliana, Voroneanu Luminita, Covic Andreea Simona, Kanbay Mehmet, Covic Adrian

机构信息

Faculty of Medicine, "Grigore T. Popa" University of Medicine and Pharmacy, 700115 Iasi, Romania.

"Dr. C.I. Parhon" Clinical Hospital, 700503 Iasi, Romania.

出版信息

Int J Mol Sci. 2025 Jun 19;26(12):5902. doi: 10.3390/ijms26125902.

Abstract

Complement system inhibitors are emerging as promising therapies in nephrology, particularly for diseases where complement dysregulation is central to pathogenesis. This review summarizes the role of complement activation in kidney diseases and current evidence supporting complement-targeted treatments. As the complement system can be involved in the pathogenesis of different diseases to varying degrees, several research works have been conducted. These research efforts aim, firstly, to understand the mechanisms and role of complement cascade components in the most prevalent nephrological diseases and, secondly, to explore the potential of complement system inhibitors in these conditions and their possible clinical applications. Clinical trials demonstrate that complement inhibitors are most effective in conditions with significant complement involvement, such as C3 glomerulopathy (C3G), atypical hemolytic uremic syndrome (aHUS), and immune complex membranoproliferative glomerulonephritis (IC-MPGN). These agents show variable benefits in diseases with partial complement activation, including lupus nephritis and ANCA-associated vasculitis, while their role in disorders like diabetic nephropathy and focal-segmental glomerulosclerosis remains limited. Complement inhibition offers a targeted strategy to prevent disease progression and improve outcomes in selected nephrological disorders.

摘要

补体系统抑制剂正在成为肾脏病学中很有前景的治疗方法,特别是对于那些补体调节异常在发病机制中起核心作用的疾病。本综述总结了补体激活在肾脏疾病中的作用以及支持补体靶向治疗的现有证据。由于补体系统可不同程度地参与不同疾病的发病机制,因此已经开展了多项研究工作。这些研究工作的目的,首先是了解补体级联成分在最常见的肾脏疾病中的机制和作用,其次是探索补体系统抑制剂在这些疾病中的潜力及其可能的临床应用。临床试验表明,补体抑制剂在补体显著参与的疾病中最有效,如C3肾小球病(C3G)、非典型溶血性尿毒症综合征(aHUS)和免疫复合物膜增生性肾小球肾炎(IC-MPGN)。这些药物在补体部分激活的疾病中显示出不同程度的益处,包括狼疮性肾炎和抗中性粒细胞胞浆抗体相关性血管炎,而它们在糖尿病肾病和局灶节段性肾小球硬化等疾病中的作用仍然有限。补体抑制为预防某些肾脏疾病的疾病进展和改善预后提供了一种靶向策略。

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