以IgA沿肾小球毛细血管襻沉积为主的急进性肾小球肾炎

目的:明确以IgA沿肾小球毛细血管襻沉积为主的急进性肾小球肾炎的临床与病理特点。方法:分析解放军总医院全军肾脏病研究所收治的1例以IgA沿肾小球毛细血管襻沉积为主的急进性肾小球肾炎病例,分析其临床特点、病理与电镜特征,以及对强化免疫抑制治疗的反应。结果:该病例临床表现为急进性肾炎综合征,肾功能恶化发展迅速。但无肺出血及全身性血管炎症状。血清自身抗体系列、抗GBM抗体与ANCA均为阴性。病理光镜示肾小球环绕型新月体形成,肾小血管无炎症改变。冰冻切片与石蜡微波修复直接免疫荧光示IgA沿肾小球毛细血管襻细颗粒状沉积,间接免疫荧光法检测血清IgA型抗GBM为阴性;电镜示节段性上皮下、基底膜内及系膜区电子致密物沉积。对强化激素冲击与免疫抑制剂治疗效果欠佳。结论:本病例为特殊类型的急进性肾小球肾炎,根据其临床特点、血清学检测、免疫荧光及电镜检查结果,无法归于目前急进性肾炎的分型,对其临床特点应予重视。

IgA deposits predominantly along glomerular basement membranes in a patient with rapidly progressive glomerulonephritis

Objective:To describe the clinical and pathological features of a rare type of rapidly progressive glomerulonephritis(RPGN) characterized as IgA deposit predominantly along glomerular basement membranes.Methods:A case with RPGN characterized as IgA deposit predominantly along glomerular basement membranes was described.The clinical and pathological features,as well as its response to treatment were analyzed.Results:The patient manifested clinically as RPGN without pulmonary hemorrhage and vasculitis related systematic symptoms.No positive results were found among ANAs,ANCAs and anti-GBM antibodies detection.Light microscopic evaluation showed circumferential crescents in more than 50 percent of the glomeruli.No vasculitis related changes were found.Direct immunofluorescence of frozen and paraffin specimens showed finely granular staining for deposits of IgA along the glomerular basement membranes.Circulating IgA anti-GBM antibodies were negative by indirect immunofluorescence detection.Electron microscopic examination showed segmental electron-dense deposits in subepithelial area and in the glomerular basement membrane.There were occasional small mesangial electron-dense deposits.Therapy with pulse methylprednisolone and intravenous cyclophosphamide was less effective.Conclusion:This case is a rare type of RPGN,which attribute hardly to the present RPGN classification.Its clinical characteristics should be noticed.