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肾小球疾病的靶向补体治疗:综述

Targeted Complement Treatments in Glomerulopathies: A Comprehensive Review.

作者信息

Gentile Micaela, Manenti Lucio

机构信息

UO Nefrologia, Dipartimento di Medicina e Chirurgia, Università di Parma, 43126 Parma, Italy.

Nephrology Unit, Azienda Sociosanitaria Liguria 5, 19121 La Spezia, Italy.

出版信息

J Clin Med. 2025 Jan 22;14(3):702. doi: 10.3390/jcm14030702.

DOI:10.3390/jcm14030702
PMID:39941374
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC11818541/
Abstract

The complement system includes soluble and cell surface proteins and is an important arm of the innate immune system. Once activated, the complement system rapidly generates proteins with inflammatory and vasoactive activities. Although complement is crucial to host defense and homeostasis, its inappropriate or uncontrolled activation can also drive tissue injury. Glomerulopathy encompasses a spectrum of diseases with diverse etiologies, clinical presentations, and outcomes. Among the intricate web of factors contributing to glomerulopathies pathogenesis, the role of complement activation has emerged as a focal point of research interest and therapeutic intervention. The pioneer drug was eculizumab, which made it possible to drastically change the prognosis of atypical hemolytic uremic syndrome, an otherwise fatal disease. This comprehensive review aims to elucidate the multifaceted interplay between complement pathways and glomerulopathies, shedding light on potential pathways for targeted therapies and improved patient care.

摘要

补体系统包括可溶性蛋白和细胞表面蛋白,是固有免疫系统的重要组成部分。一旦被激活,补体系统会迅速产生具有炎症和血管活性的蛋白质。虽然补体对于宿主防御和内环境稳定至关重要,但其不适当或不受控制的激活也会导致组织损伤。肾小球病包括一系列病因、临床表现和预后各异的疾病。在导致肾小球病发病机制的复杂因素网络中,补体激活的作用已成为研究兴趣和治疗干预的焦点。先驱药物是依库珠单抗,它使彻底改变非典型溶血性尿毒症综合征(一种原本致命的疾病)的预后成为可能。这篇综述旨在阐明补体途径与肾小球病之间多方面的相互作用,为靶向治疗的潜在途径和改善患者护理提供线索。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9653/11818541/434d21a852fb/jcm-14-00702-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9653/11818541/434d21a852fb/jcm-14-00702-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9653/11818541/434d21a852fb/jcm-14-00702-g001.jpg

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引用本文的文献

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J Clin Med. 2025 Apr 7;14(7):2527. doi: 10.3390/jcm14072527.

本文引用的文献

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Alternative Complement Pathway Inhibition with Iptacopan in IgA Nephropathy.Iptacopan对替代补体途径的抑制作用在IgA肾病中的研究
N Engl J Med. 2025 Feb 6;392(6):531-543. doi: 10.1056/NEJMoa2410316. Epub 2024 Oct 25.
2
Safety and Efficacy of Avacopan in Patients with Complement 3 Glomerulopathy: Randomized, Double-Blind Clinical Trial.阿伐可泮治疗补体3肾小球病患者的安全性和有效性:随机双盲临床试验
J Am Soc Nephrol. 2025 Mar 1;36(3):487-499. doi: 10.1681/ASN.0000000526. Epub 2024 Oct 11.
3
Therapy of IgA nephropathy: time for a paradigm change.
IgA肾病的治疗:是时候进行范式转变了。
Front Med (Lausanne). 2024 Aug 15;11:1461879. doi: 10.3389/fmed.2024.1461879. eCollection 2024.
4
Real-world safety profile of eculizumab: an analysis of FDA adverse event reporting system and systematic review of case reports.依库珠单抗的真实世界安全性概况:对美国食品药品监督管理局不良事件报告系统的分析及病例报告的系统评价
Expert Opin Drug Saf. 2024 Aug 26:1-7. doi: 10.1080/14740338.2024.2392885.
5
The role of complement in kidney disease: conclusions from a Kidney Disease: Improving Global Outcomes (KDIGO) Controversies Conference.补体在肾脏疾病中的作用:KDIGO(改善全球肾脏病预后组织)争议会议的结论。
Kidney Int. 2024 Sep;106(3):369-391. doi: 10.1016/j.kint.2024.05.015. Epub 2024 Jun 4.
6
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J Clin Med. 2024 Apr 28;13(9):2594. doi: 10.3390/jcm13092594.
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