Departments of Internal Medicine and Pediatrics, University of Iowa, Iowa City, Iowa, USA.
Curr Opin Nephrol Hypertens. 2013 Mar;22(2):231-7. doi: 10.1097/MNH.0b013e32835da24c.
The purpose of this review is to discuss emerging nomenclature, review the salient clinicopathological features and describe the therapeutic options available for the treatment of C3 glomerulopathy (C3G).
C3G is minimally responsive to traditional immune suppression and randomized controlled trials to support therapy are absent. The burgeoning understanding of the role of the alternative complement pathway in C3G combined with animal data supporting the use of terminal complement blockade and a few reports suggesting that the anticomplement drug eculizumab may offer a therapeutic advantage have triggered great interest in the field of complement-mediated renal disease.
Anticellular immune suppression and plasma therapy have limited efficacy in C3G. Data suggest that eculizumab may ameliorate disease in some C3G patients. The limited, recently published cohort data highlight crucial aspects of this group of diseases and support the need for extensive genetic and biomarker research to validate the pathologic mechanisms, delineate the spectrum of disease and guide the design of the rigorous trials to identify effective therapies for the treatment of C3G.
本文旨在讨论新兴命名法，回顾 C3 肾小球病（C3G）的显著临床病理特征，并描述其治疗方法。
C3G 对传统免疫抑制的反应性较差，且缺乏支持治疗的随机对照试验。越来越多的证据表明替代补体途径在 C3G 中的作用，结合动物数据支持使用末端补体阻断，以及一些报告表明抗补体药物依库珠单抗可能具有治疗优势，这激发了人们对补体介导的肾脏疾病领域的极大兴趣。
抗细胞免疫抑制和血浆疗法在 C3G 中的疗效有限。数据表明，依库珠单抗可能改善某些 C3G 患者的疾病。最近发表的有限队列数据突出了这类疾病的关键方面，并支持需要广泛的遗传和生物标志物研究来验证病理机制、描述疾病谱，并指导严格的试验设计，以确定治疗 C3G 的有效疗法。
