Atypical anti-glomerular basement membrane disease superimposed on IgA nephropathy

T. Balasubramaniyan, J. Dhanapriya, T. Dineshkumar, A. T. Maasila, S. Arivazhagan, D. Rajasekar, R. Sakthirajan, N. Gopalakrishnan
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引用次数: 2

Abstract

Anti-glomerular basement membrane (anti-GBM) disease is an autoimmune disorder characterized by crescentic glomerulonephritis, pulmonary hemorrhage, and the presence of circulating anti-GBM antibodies which bind to the α3 chain of Type IV collagen found in the GBM. IgA nephropathy (IgAN) is the most common form of primary glomerulonephritis worldwide. The simultaneous occurrence of atypical anti-GBM disease and IgAN has not been reported previously. We report here two female patients who presented with oliguria, hypertension, and renal failure. Renal biopsy revealed crescentic glomerulonephritis and bright linear IgG staining along glomerular capillary walls and mesangial IgA (3+) deposits in immunofluorescence. Serology was negative for anti-GBM antibodies both by ELISA and immunoblot assays. Hence, a diagnosis of atypical anti-GBM disease with superimposed IgAN was made. Both patients were treated with hemodialysis, intravenous steroids, and cyclophosphamide with the improvement of renal function in one patient and the other progressed to end-stage renal disease.
IgA肾病合并非典型抗肾小球基底膜病
抗肾小球基底膜病(anti-GBM)是一种自身免疫性疾病,其特征是月牙状肾小球肾炎、肺出血和循环中存在与GBM中IV型胶原α3链结合的抗GBM抗体。IgA肾病(IgAN)是世界范围内最常见的原发性肾小球肾炎。非典型抗gbm疾病和IgAN同时发生的报道以前没有报道过。我们在此报告两名女性患者,她们表现为少尿、高血压和肾衰竭。肾活检显示月牙状肾小球肾炎,免疫荧光显示肾小球毛细血管壁有明亮的线性IgG染色,系膜有IgA(3+)沉积。ELISA和免疫印迹检测血清抗gbm抗体均为阴性。因此,诊断非典型抗gbm疾病与叠加IgAN作出。两例患者均接受血液透析、静脉注射类固醇和环磷酰胺治疗,其中一例患者肾功能改善,另一例进展为终末期肾病。
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