ISSN 1006-298X      CN 32-1425/R

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肾脏病与透析肾移植杂志 ›› 2018, Vol. 27 ›› Issue (4): 301-305.DOI: 10.3969/j.issn.1006-298X.2018.04.001

• 论文 •    下一篇

232例膜增生性肾小球肾炎的重新评估

  

  • 出版日期:2018-08-28 发布日期:2018-08-31

Reevaluation of 232 cases of membranoproliferative glomerulonephritis

  • Online:2018-08-28 Published:2018-08-31

摘要:

目的:探讨膜增生性肾小球肾炎(membranoproliferative glomerulonephritis,MPGN)的重新评估。
方法:收集2011年1月至2016年9月南京总医院国家肾脏疾病临床医学研究中心肾活检光镜下表现为MPGN患者(排除狼疮性肾炎)的临床病理资料,根据免疫荧光、电镜和可能病因并结合Mayo/RPS对其重新进行分类研究。
结果:232例MPGN患者,35例(151%)为C3肾小球病,197例(849%)为免疫复合物(Ig+/-C3)介导的MPGN。后者包括55例肾组织单克隆免疫球蛋白沉积的MPGN和142例多克隆沉积的MPGN。单克隆沉积的MPGN进一步分为44例伴单克隆免疫球蛋白Ig沉积的增生性肾小球肾炎和11例单克隆免疫球蛋白沉积病;多克隆沉积的MPGN依次分为52例感染相关(乙肝/丙肝病毒感染)、4例自身免疫相关,以及22例冷球蛋白相关、1例纤维性肾小球肾炎,63例特发性MPGN。生存分析显示单克隆沉积的MPGN肾脏存活中位时间为42个月,显著短于多克隆组(P=0013)。
结论:(1)基于免疫荧光的MPGN新分类对于揭示致病机制、寻找病因、判断预后有实用价值;(2)单克隆免疫球蛋白沉积和肝炎病毒感染是我国继发性MPGN的第主要病因。

 

关键词: 膜増生性肾小球肾炎, 新分类, 肾脏意义的单克隆免疫球蛋白

Abstract:

Objective:To investigate the new classification of membranoproliferative glomerulonephritis (MPGN).
Methodology:The clinicopathological data of MPGN patients (excluding lupus nephritis) diagnosed by renal biopsy in Nanjing General Hospital from January 2011 to September 2016 were collected and classified according to the new immunofluorescence classification proposed by Mayo Clinic.
Results:Among the 232 patients with MPGN,35(151%)were diagnosed with C3 Glomerulopathy and 197(849%)were diagnosed with immune complexmediated MPGN.The latter composed of 55 patients with monoclonal deposits in the kidney which could be further divided in 44 patients with proliferative glomerulonephritis with monoclonal immunoglobulin deposits and 11 patients with renal monoclonal immunoglobulin deposition disease,and 142 patients with polyclonal deposits including 52 infectionrelated cases(HBV/HCV),4 autoimmune related cases,22 cryoglobulinemic related cases,1 case fibrillary glomerulonephritis and 63 cases Idiopathic.The renal survival curve revealed that median survival time was 42 months in monoclonal group,significantly shorter than in  polyclonal group (P=0013).
Conclusion:(1) The new classification of MPGN based on immunofluorescence has practical value in revealing pathogenesis,finding cause and judging prognosis; (2) Monoclonal immunoglobulinopathy and hepatitis virus infection were the first and second etiology of secondary MPGN in our country.

Key words: membraneproliferative glomerulonephritis, new classification, membranoproliferative glomerulonephritis