补体系统调控异常与C3肾小球病

Complement system regulation and C3 glomerulopathy

目前研究发现，越来越多的累及肾的炎症性疾病与补体系统渊节异常有关，如不典型溶血尿毒综合征（a—typical hemolytic uremic syndrome，aHUS）、中性粒细胞胞浆抗体（antineutrophil cytoplasmic an—tibodies，ANCA）相关性小血管炎及儿童期起病的系统性红斑狼疮等，而各种原发性肾小球疾病与补体调节异常关系更为密切，如致密物沉积病（dense de—posit disease，DDD）和C3肾小球肾炎等。

Complement system is a key system for immune surveillance and homeostasis. Excessive ac-tivation 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 glomerulopa-thy.