单克隆免疫球蛋白相关C3肾小球肾炎研究进展

Recent advances in C3 glomerulopathy with monoclonal gammopathy

单克隆免疫球蛋白相关C3肾小球肾炎(C3 glomerulopathy with monoclonal gammopathy,C3G-MIg)是一种罕见的单克隆免疫球蛋白病,是50岁以上患者肾损伤的重要病因。C3GMIg关键的发病机制是单克隆免疫球蛋白作为自身抗体或通过直接裂解C3的方式,过度激活补体旁路途径,导致补体沉积进而损伤肾脏。针对产生单克隆免疫球蛋白的B细胞或浆细胞及过度激活的补体旁路途径,C3G-MIg的治疗可采取支持治疗、化疗、克隆抗体联合治疗的方法。本文回顾了有关C3G-MIg的研究,综述其发病机制、临床表现及治疗进展,为临床诊治C3G-MIg提供新思路。

As a rare monoclonal gammopathy,C3 glomerulopathy with monoclonal gammopathy(C3G-MIg)is a major cause of renal injury in patients aged over 50 years.The key pathogenesis of C3GMIg is that monoclonal immunoglobulins act as an autoantibody or directly clefts complement C3 to excessively activate the alternative pathway,leading to complement deposition and further injuring kidneys.For B cells or plasma cells producing monoclonal immunoglobulins and over-activating alternative pathway,proper treatments of C3G-MIg include a combination of supportive cares,chemotherapy and clonal antibody therapy.Focusing upon researches on C3G-MIg,this review summarized its pathogenetic mechanisms,clinical manifestations and treatment advances,providing new therapeutic rationales for C3G-MIg.