IgA mediated anti-glomerular basement membrane disease with associated circulating anti-neutrophil cytoplasm antibodies

Q4 Medicine Journal of Nephropathology Pub Date : 2023-02-17 DOI:10.34172/jnp.2023.17371
P. Alves, M. Góis, Tiago Pereira, D. Carvalho, F. Nolasco
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Abstract

Anti-glomerular basement membrane (anti-GBM) disease is an aggressive small vessel vasculitis usually mediated by IgG autoantibodies. We describe the case of a 73-year-old male with rapidly progressive renal failure that was diagnosed with IgA mediated anti-GBM disease associated with circulating anti-neutrophil cytoplasm antibodies (ANCA), where the diagnosis was established on kidney biopsy by detecting linear deposition of IgA along the GBM on immunofluorescence microscopy. Despite an intensive immunosuppressive regimen with plasmapheresis, steroids and oral cyclophosphamide, the disease progressed to end-stage renal failure and the patient was started on hemodialysis.
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IgA介导的抗肾小球基底膜疾病与相关的循环抗中性粒细胞细胞质抗体
抗肾小球基底膜病(anti-GBM)是一种侵袭性小血管炎,通常由IgG自身抗体介导。我们描述了一个73岁的男性快速进展性肾衰竭的病例,他被诊断为IgA介导的抗GBM疾病,与循环抗中性粒细胞细胞质抗体(ANCA)相关,通过在免疫荧光显微镜下检测IgA沿GBM的线性沉积,在肾活检中建立了诊断。尽管患者接受了血浆置换、类固醇和口服环磷酰胺的强化免疫抑制治疗,但病情进展为终末期肾衰竭,患者开始进行血液透析。
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来源期刊
Journal of Nephropathology
Journal of Nephropathology Medicine-Nephrology
CiteScore
1.30
自引率
0.00%
发文量
35
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