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抗肾小球基底膜病与IgA肾病并存:一例说明性病例及文献综述

Coexistence of anti-glomerular basement membrane disease and IgA nephropathy: an illustrative case and comprehensive literature review.

作者信息

Chen Zewei, Xu Dechao, Cui Fangzheng, Hou Huihui, Mao Zhiguo, Gao Xiang

机构信息

Kidney Institute, Department of Nephrology, Shanghai Changzheng Hospital, Naval Military Medical University, Shanghai, China.

Department of Nephrology, The First Navy Hospital of Southern Theater Command, Zhanjiang, China.

出版信息

Ren Fail. 2024 Dec;46(1):2323160. doi: 10.1080/0886022X.2024.2323160. Epub 2024 Mar 11.

Abstract

Anti-glomerular basement membrane (GBM) disease is a rare autoimmune condition characterized by the presence of positive anti-GBM autoantibodies, linear deposition of immunoglobulin G (IgG) along the GBM and severe kidney injury. In a limited number of cases, the association of anti-GBM disease with other glomerulonephritis has been reported. Herein, we present the case of a 66-year-old female patient with progressive worsen kidney function and decreased urine output. A renal biopsy revealed crescent glomerulonephritis with lineal IgG deposition along the GBM and mesangial IgA deposition, which supported the diagnosis of concurrent anti-GBM disease and IgA nephropathy (IgAN). In an extensive literature review, we identified a total of thirty-nine patients were reported anti-GBM disease combined with IgAN. The clinical characteristics of these patients demonstrate that the anti-GBM disease combined with IgAN tends to be milder with a more indolent course and a better prognosis than the classic anti-GBM disease, and its potential pathogenesis deserves to be further explored.

摘要

抗肾小球基底膜(GBM)病是一种罕见的自身免疫性疾病,其特征为存在阳性抗GBM自身抗体、免疫球蛋白G(IgG)沿GBM呈线性沉积以及严重肾损伤。在少数病例中,已有抗GBM病与其他肾小球肾炎相关联的报道。在此,我们报告一例66岁女性患者,其肾功能进行性恶化,尿量减少。肾活检显示新月体性肾小球肾炎,沿GBM有线性IgG沉积及系膜IgA沉积,这支持了同时存在抗GBM病和IgA肾病(IgAN)的诊断。在广泛的文献综述中,我们共确定了39例报告的抗GBM病合并IgAN患者。这些患者的临床特征表明,与经典抗GBM病相比,抗GBM病合并IgAN往往病情较轻,病程更隐匿,预后更好,其潜在发病机制值得进一步探索。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8ce1/10930099/603523ec70fc/IRNF_A_2323160_F0001_C.jpg

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