重视C3肾小球病的诊断与鉴别诊断

Pay attention to the diagnosis and differential diagnosis of C3 glomerulopathy

C3肾小球病是新近被认识的一类肾小球疾病,2015年被纳入梅奥最新肾小球肾炎病理分型之列,足见其重要性。该病最显著的特点是免疫荧光染色肾小球以补体C3沉积为主,先天或获得性补体调节缺陷致补体旁路途径异常活化是其主要发病机制。及时正确的诊断是临床治疗决策及改善预后的关键。文章将重点介绍C3肾小球病的发病机制、诊断及鉴别诊断,以期提高临床对C3肾小球病的诊断和治疗水平。

C3 glomerulopathy is a recently introduced disease, and has drawn attention of renal pathologists and nephrologists. The characteristic histological feature is the presence of dominant C3 staining in the glomerulus by immunofluorescence staining. Abnormal activation of complement alternative pathway caused by congenital or acquired complement regulation deficiency is the main pathogenesis of C3 glomerulopathy. Timely and precise diagnosis to treatment decisions and improvement of disease outcome in patients with C3 glomerulopathy. The authors will focus on the pathogenesis and diagnosis of the disease in this review, hoping to improve the level of diagnosis and treatment.