Xiao Hui-jie, He Rui-juan
Department of Pediatrics, Peking University First Hospital, Beijing 100034, China.
Beijing Da Xue Xue Bao Yi Xue Ban. 2013 Apr 18;45(2):323-6.
Complement system is a key system for immune surveillance and homeostasis. Excessive activation of complement system,especially the activation of alternative pathway may play a very important role in the pathogenesis of primary and secondary glomerulonephritis. C3 glomerulopathy is a newly named disease characterized by evident C3 deposition in the glomeruli with little or no immunoglobulin under immunofluorescence (IF). Its clinical and pathological manifestations vary a lot. The decreased plasma C3 and Factor H(FH)suggest that abnormal regulation of complement system plays an importment role in its pathogenesis. C3 glomerulopathy varies a lot as to its clinical manifestation, treatment and prognosis. The inhibition of excessive complement activation might be the key to treating C3 glomerulopathy.
补体系统是免疫监视和内环境稳定的关键系统。补体系统的过度激活,尤其是替代途径的激活,可能在原发性和继发性肾小球肾炎的发病机制中起非常重要的作用。C3肾小球病是一种新命名的疾病,其特征是在免疫荧光(IF)下肾小球内有明显的C3沉积,而免疫球蛋白很少或没有。其临床和病理表现差异很大。血浆C3和H因子(FH)降低表明补体系统的异常调节在其发病机制中起重要作用。C3肾小球病在临床表现、治疗和预后方面差异很大。抑制补体过度激活可能是治疗C3肾小球病的关键。