Giaime Philippe, Daniel Laurent, Burtey Stéphane
Clin Nephrol. 2015 Jan;83(1):57-60. doi: 10.5414/CN107945.
C3 glomerulopathy (C3GP) is a membranoproliferative glomerulonephritis without immunoglobulin deposits. Activation of the alternative pathway of the complement system is central in its pathogenesis. The presence of a C3 nephritic factor (C3Nef), or deficient factor H or I is associated with C3 GP. The treatment is not codified. Here we describe a case of a Caucasian male with a dense deposit disease (a form of C3GP) revealed by nephritic syndrome. C3Nef was present. We treated him with rituximab as the sole immunosuppressive regimen and obtained a complete remission on proteinuria. The effect was sustained at more than 2 years with only two courses of treatment and an excellent tolerance. Rituximab could be proposed as a treatment of C3GP associated with antibodies interfering with complement alternative pathway.
C3肾小球病(C3GP)是一种无免疫球蛋白沉积的膜增生性肾小球肾炎。补体系统替代途径的激活在其发病机制中起核心作用。C3肾炎因子(C3Nef)的存在、因子H或I的缺乏与C3GP相关。治疗方法尚无统一规范。在此,我们描述一例白种男性患者,因肾病综合征发现患有致密物沉积病(C3GP的一种形式)。该患者存在C3Nef。我们仅使用利妥昔单抗作为免疫抑制方案对其进行治疗,蛋白尿完全缓解。仅经过两个疗程的治疗,疗效持续超过2年,且耐受性良好。对于与干扰补体替代途径的抗体相关的C3GP,可考虑使用利妥昔单抗进行治疗。