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局灶节段性肾小球硬化的病理生理学与治疗进展:及时且个性化治疗方法的重要性

Advances in the pathophysiology and treatment of focal segmental glomerulosclerosis: The importance of a timely and tailored approach.

作者信息

Gembillo Guido, Sessa Concetto, Santoro Domenico

机构信息

Unit of Nephrology and Dialysis, AOU "G. Martino", University of Messina, Messina 98125, Sicilia, Italy.

Unit of Nephrology and Dialysis, P.O. Maggiore "Nino Baglieri", Ragusa 97100, Sicilia, Italy.

出版信息

World J Nephrol. 2025 Jun 25;14(2):103039. doi: 10.5527/wjn.v14.i2.103039.

DOI:10.5527/wjn.v14.i2.103039
PMID:40568339
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC12001220/
Abstract

Focal segmental glomerulosclerosis (FSGS) is a histological pattern of glomerular damage that significantly contributes to chronic kidney disease and end-stage renal disease. Its incidence is rising globally, necessitating timely and personalized management strategies. This paper aims to provide an updated overview of the pathophysiology, diagnosis, and therapeutic strategies for FSGS, emphasizing the importance of early interventions and tailored treatments. This editorial synthesizes key findings from recent literature to highlight advancements in understanding and managing FSGS. Emerging evidence supports the role of targeted therapies and personalized approaches in improving outcomes for FSGS patients. Advances include novel biomarkers, genetic testing, and innovative therapeutics such as transient receptor potential ion channel blockers and antisense oligonucleotides for apolipoprotein 1-related FSGS. Effective management of FSGS requires a combination of timely diagnosis, evidence-based therapeutic strategies, and ongoing research to optimize patient outcomes and address gaps in the current understanding of the disease.

摘要

局灶节段性肾小球硬化(FSGS)是一种肾小球损伤的组织学模式,对慢性肾脏病和终末期肾病有显著影响。其全球发病率正在上升,因此需要及时且个性化的管理策略。本文旨在提供FSGS病理生理学、诊断及治疗策略的最新综述,强调早期干预和个性化治疗的重要性。这篇社论综合了近期文献的关键发现,以突出在FSGS理解和管理方面的进展。新出现的证据支持靶向治疗和个性化方法在改善FSGS患者预后中的作用。进展包括新型生物标志物、基因检测以及诸如瞬时受体电位离子通道阻滞剂和载脂蛋白1相关FSGS的反义寡核苷酸等创新疗法。FSGS的有效管理需要及时诊断、基于证据的治疗策略以及持续研究相结合,以优化患者预后并填补当前对该疾病认识的空白。

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Advances in the pathophysiology and treatment of focal segmental glomerulosclerosis: The importance of a timely and tailored approach.局灶节段性肾小球硬化的病理生理学与治疗进展:及时且个性化治疗方法的重要性
World J Nephrol. 2025 Jun 25;14(2):103039. doi: 10.5527/wjn.v14.i2.103039.
2
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本文引用的文献

1
Clinical course and outcome of adult patients with primary focal segmental glomerulosclerosis with kidney function loss on presentation.初诊时伴有肾功能丧失的成人原发性局灶节段性肾小球硬化症患者的临床病程及预后
World J Nephrol. 2024 Dec 25;13(4):98932. doi: 10.5527/wjn.v13.i4.98932.
2
Antinephrin-Associated Primary Focal Segmental Glomerulosclerosis Successfully Treated With Plasmapheresis.血浆置换成功治疗抗肾素相关原发性局灶节段性肾小球硬化症
Kidney Int Rep. 2024 Jul 1;9(9):2829-2831. doi: 10.1016/j.ekir.2024.06.038. eCollection 2024 Sep.
3
Focal Segmental Glomerulosclerosis.局灶节段性肾小球硬化症。
Adv Kidney Dis Health. 2024 Jul;31(4):275-289. doi: 10.1053/j.akdh.2024.03.009.
4
Focal Segmental Glomerulosclerosis Patient Baseline Characteristics in the Sparsentan Phase 3 DUPLEX Study.司帕生坦3期双盲研究中局灶节段性肾小球硬化症患者的基线特征
Kidney Int Rep. 2024 Jan 28;9(4):1020-1030. doi: 10.1016/j.ekir.2024.01.032. eCollection 2024 Apr.
5
Differentiating primary and secondary FSGS using non-invasive urine biomarkers.使用非侵入性尿液生物标志物鉴别原发性和继发性局灶节段性肾小球硬化症。
Clin Kidney J. 2023 Dec 4;17(2):sfad296. doi: 10.1093/ckj/sfad296. eCollection 2024 Feb.
6
Perihilar FSGS lesions originate from flat parietal epithelial cells.肾门周围局灶节段性肾小球硬化病变起源于扁平壁层上皮细胞。
J Nephrol. 2024 Jun;37(5):1405-1409. doi: 10.1007/s40620-024-01886-y. Epub 2024 Feb 1.
7
A multi-institutional study found a possible role of anti-nephrin antibodies in post-transplant focal segmental glomerulosclerosis recurrence.一项多机构研究发现抗肾素抗体在移植后局灶节段性肾小球硬化复发中的可能作用。
Kidney Int. 2024 Mar;105(3):608-617. doi: 10.1016/j.kint.2023.11.022. Epub 2023 Dec 16.
8
Cure Glomerulonephropathy Pathology Classification and Core Scoring Criteria, Reproducibility, and Clinicopathologic Correlations.治愈性肾小球肾病的病理分类及核心评分标准、可重复性和临床病理相关性
Glomerular Dis. 2023 Oct 26;3(1):248-257. doi: 10.1159/000534755. eCollection 2023 Jan-Dec.
9
Current understanding of the molecular mechanisms of circulating permeability factor in focal segmental glomerulosclerosis.目前对局灶节段性肾小球硬化症中循环通透因子的分子机制的认识。
Front Immunol. 2023 Sep 19;14:1247606. doi: 10.3389/fimmu.2023.1247606. eCollection 2023.
10
A Study of Focal and Segmental Glomerulosclerosis according to the Columbia Classification and Its Correlation with the Clinical Outcome.基于哥伦比亚分类法的局灶节段性肾小球硬化症研究及其与临床结局的相关性
J Lab Physicians. 2023 Feb 24;15(3):431-436. doi: 10.1055/s-0043-1761930. eCollection 2023 Sep.