补体系统在C3肾小球病中的作用

摘要

Objective C3 glomerulopathy describes a spectrum of disorders with glomerular pathology associated with C3 cleavage product deposition and with defective complement action and regulation. Kidney biopsies from these patients show glomerular accumulation or deposition of C3 cleavage fragments, but no or minor deposition of immunoglobulins.C3G is subdivided into C3G-DDD with specific dense deposits subforms and into C3 glomerulonephritis. C3G is considered to be the consequences of defective complement control and in particular deregulation of the alternative pathway, and complement system is associated with genetic variations and autoimmune factors. At present further studies are needed to clear the pathogenesis of the disease, identify the precise biological markers and develop effective treatments.%C3肾小球病是一组与C3裂解产物沉积及补体缺陷和/或失调相关的肾小球病。其肾活检特征性表现为免疫荧光染色C3沉积为主，不伴或伴少量其他免疫复合物沉积。C3肾小球病被认为是补体调控缺陷及旁路途径过度活化的结果，而补体调控异常与遗传变异和自身免疫有关。目前尚需进一步研究明确疾病的发病机制、找出精确的生物学标志物以及制定有效的治疗方法。