Conversano Ester, Vivarelli Marina
Division of Nephrology, Bambino Gesù Children's Hospital IRCCS, 00165 Rome, Italy.
Laboratory of Nephrology and Clinical Trial Center, Bambino Gesù Children's Hospital IRCCS, 00165 Rome, Italy.
J Clin Med. 2025 Jun 13;14(12):4204. doi: 10.3390/jcm14124204.
There is rapidly increasing evidence of the role of complement in different forms of kidney disease and this has broadened the field to involve not only atypical hemolytic uremic syndrome (aHUS) and C3 glomerulopathy (C3G), but also a number of other glomerular diseases, mainly ANCA-associated renal vasculitis, immune-complex glomerulonephritis, membranous nephropathy, and IgA nephropathy (IgAN). In parallel, the field of therapeutic agents able to target the three complement pathways at different levels, both proximally and terminally, has grown tremendously in recent years. This has led to the approval of agents targeting complement for ANCA-associated vasculitis, IgA nephropathy, and, very recently, C3 glomerulopathy. The real-world implementation of these agents remains a challenge. This review will attempt, through the presentation of representative clinical vignettes, to provide some practical guidance for the nephrologist in how to navigate these new therapeutic opportunities, focusing on aHUS, C3G, and IgAN.
越来越多的证据表明补体在不同形式的肾脏疾病中发挥作用，这拓宽了该领域的范围，不仅涉及非典型溶血性尿毒症综合征（aHUS）和C3肾小球病（C3G），还包括许多其他肾小球疾病，主要是抗中性粒细胞胞浆抗体相关性肾血管炎、免疫复合物肾小球肾炎、膜性肾病和IgA肾病（IgAN）。与此同时，近年来能够在不同水平（近端和终末）靶向三条补体途径的治疗药物领域有了巨大发展。这导致了针对抗中性粒细胞胞浆抗体相关性血管炎、IgA肾病以及最近针对C3肾小球病的补体靶向药物获批。这些药物在现实世界中的应用仍然是一项挑战。本综述将试图通过呈现具有代表性的临床案例，为肾病学家提供一些实用指导，以帮助他们把握这些新的治疗机会，重点关注aHUS、C3G和IgAN。
