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Atypical postinfectious glomerulonephritis is associated with abnormalities in the alternative pathway of complement

发布于：2013年8月5日    文字：【大】【中】【小】

Kidney Int. 2013 Feb;83(2):293-9. doi: 10.1038/ki.2012.384. Epub 2012 Dec 12.

Atypical postinfectious glomerulonephritis is associated with abnormalities in the alternative pathway of complement.

Sethi S, Fervenza FC, Zhang Y, Zand L, Meyer NC, Borsa N, Nasr SH, Smith RJ.

Source

Division of Anatomic Pathology, Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, Minnesota 55905, USA. Sethi.sanjeev@mayo.edu

Abstract

Postinfectious glomerulonephritis is a common disorder that develops following an infection. In the majority of cases, there is complete recovery of renal function within a few days to weeks following resolution of the infection. In a small percentage of patients, however, the glomerulonephritis takes longer to resolve, resulting in persistent hematuria and proteinuria, or even progression to end-stage kidney disease. In some cases of persistent hematuria and proteinuria, kidney biopsies show findings of a postinfectious glomerulonephritis even in the absence of any evidence of a preceding infection. The cause of such 'atypical' postinfectious glomerulonephritis, with or without evidence of preceding infection, is unknown. Here we show that most patients diagnosed with this 'atypical' postinfectious glomerulonephritis have an underlying defect in the regulation of the alternative pathway of complement.These defects include mutations in complement-regulating proteins and antibodies to the C3 convertase known as C3 nephritic factors. As a result, the activated alternative pathway is not brought under control even after resolution of the infection. Hence, the sequela is continual glomerular deposition of complement factors with resultant inflammation and development of an 'atypical' postinfectious glomerulonephritis.

感染后肾小球肾炎是一种常见的由感染导致的疾病。大多数患者在感染被控制后的几天内，肾功能可以得到完全的恢复。但是有少数的患者肾功能的恢复需要较长的时间，造成持续的血尿和蛋白尿，甚至进展至终末期肾脏病。在一些持续血尿和蛋白尿的患者，甚至在没有前驱感染证据患者的肾脏穿刺中可以发现感染后肾小球肾炎的证据。这种“不典型”感染后肾小球肾炎，无论是否存在前驱感染证据，其病因目前都还不明确。这里我们发现大多数“不典型”感染后肾小球肾炎的患者都存在补体旁路途径调节的紊乱。这些患者中存在补体调节蛋白基因的缺陷或者C3肾炎因子的存在。造成的后果是活化的补体旁路在感染恢复后也处于失调状态。因此，在肾脏局部持续的补体成分的沉积可能造成了这种“不典型”感染后肾小球肾炎的发生。