C3 肾小球病的补体末端通路激活和肾小球内免疫反应

IF 10.3 1区 医学 Q1 UROLOGY & NEPHROLOGY Journal of The American Society of Nephrology Pub Date : 2024-08-01 Epub Date: 2024-05-06 DOI:10.1681/ASN.0000000000000373
Marie-Sophie Meuleman, Florent Petitprez, Matthew C Pickering, Moglie Le Quintrec, Mikel Rezola Artero, Anna Duval, Marion Rabant, Alyssa Gilmore, Olivia Boyer, Julien Hogan, Aude Servais, François Provot, Vivianne Gnemmi, Maeva Eloudzeri, Anne Grunenwald, David Buob, Jean-Jacques Boffa, Anissa Moktefi, Vincent Audard, Jean-Michel Goujon, Frank Bridoux, Eric Thervet, Alexandre Karras, Lubka T Roumenina, Véronique Frémeaux Bacchi, Jean-Paul Duong Van Huyen, Sophie Chauvet
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引用次数: 0

摘要

背景C3肾小球病是一种因补体替代途径过度激活而导致的罕见疾病。虽然也有证据表明存在终末途径激活,但对其发生和对疾病的影响研究甚少:我们回顾性研究了42例被诊断为C3肾小球病变的患者。我们对组织学参数进行了集中、广泛的特征描述。肾脏 C5b-9 染色是终末通路激活的标志物,肾内免疫反应的特征则通过转录组分析来确定:结果:88%的活检组织显示肾小球中有C5b-9沉积。根据C5b-9沉积量对活检样本进行分组(无或低沉积n=15/42,占36%;中等沉积n=15/42,占36%;高沉积n=12/42,占28%)。C5b-9沉积较高的患者与其他两组患者有显著差异,组织学慢性化评分显著较高(P=0.005),无结果生存率较低(P=0.001)。在多变量分析中,肾小球C5b-9越高,肾脏预后越差。与对照组相比,所研究的847个免疫基因中有三分之一在C3肾小球病活检中上调。对差异表达基因的无监督聚类确定了一组肾活检样本富含高肾小球C5b-9,具有高免疫和成纤维特征,并在组织学检查中显示出高慢性化评分:结论:在一组C3肾小球病变患者中,肾脏内终末通路激活与特定的组织学表型和疾病预后有关。
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Complement Terminal Pathway Activation and Intrarenal Immune Response in C3 Glomerulopathy.
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来源期刊
Journal of The American Society of Nephrology
Journal of The American Society of Nephrology 医学-泌尿学与肾脏学
CiteScore
22.40
自引率
2.90%
发文量
492
审稿时长
3-8 weeks
期刊介绍: The Journal of the American Society of Nephrology (JASN) stands as the preeminent kidney journal globally, offering an exceptional synthesis of cutting-edge basic research, clinical epidemiology, meta-analysis, and relevant editorial content. Representing a comprehensive resource, JASN encompasses clinical research, editorials distilling key findings, perspectives, and timely reviews. Editorials are skillfully crafted to elucidate the essential insights of the parent article, while JASN actively encourages the submission of Letters to the Editor discussing recently published articles. The reviews featured in JASN are consistently erudite and comprehensive, providing thorough coverage of respective fields. Since its inception in July 1990, JASN has been a monthly publication. JASN publishes original research reports and editorial content across a spectrum of basic and clinical science relevant to the broad discipline of nephrology. Topics covered include renal cell biology, developmental biology of the kidney, genetics of kidney disease, cell and transport physiology, hemodynamics and vascular regulation, mechanisms of blood pressure regulation, renal immunology, kidney pathology, pathophysiology of kidney diseases, nephrolithiasis, clinical nephrology (including dialysis and transplantation), and hypertension. Furthermore, articles addressing healthcare policy and care delivery issues relevant to nephrology are warmly welcomed.
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