C3肾小球病和膜增生性肾小球肾炎

C3 glomerulopathy and membranoproliferative glomerulonephritis

C3肾小球病(C3 glomerulopathy,C3G)是一个基于临床、病理和病因的诊断术语,指一类主要继发于血浆补体替代途径和肾小球微环境失调引起的疾病谱。膜增生性肾小球肾炎(membranoproliferative glomerulonephritis,MPGN)是肾活检光镜下病理改变之一而非某一特种疾病或诊断术语。C3G的光镜病理类型主要为MPGN,是MPGN的主要原发病因之一。本文就C3G和MPGN的病因、临床表现、病理特征等进行综述,特别强调的是C3G可以作为临床第一诊断或主要诊断,而MPGN仅仅是一种光镜病理表现而非某种特定疾病,不建议作为临床第一诊断或主要诊断,在肾活检提示为MPGN病变时,应积极寻求其原发病因以明确诊断。

As a clinical,etiological and pathological diagnosis,C3 glomerulopathy(C3G)refers to a diverse group of rare kidney diseases characterized by complement dysregulation in fluid phase and glomerular microenvironment.As a non-specific condition,membranoproliferative glomerulonephritis(MPGN)is one of the pathological changes of renal biopsy under light microscope.The light microscopic pathological change of C3G is predominantly membranous MPGN while C3G is a major cause of MPGN.This review summarized the etiologies,clinical manifestations and pathological features of C3G and MPGN.C3G may be used as an initial or a major clinical diagnosis while MPGN is merely a light microscopic pathological change.When renal biopsy hints at MPGN,primary etiology should be actively sought for a definite diagnosis.