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以节段性模式伴孤立性免疫球蛋白A沉积的膜性肾病:一例报告

Membranous nephropathy as a segmental pattern with solitary immunoglobulin A deposition: a case report.

作者信息

Yokote Shinya, Hatanaka Saeko, Shimizu Akihiro, Okabe Masahiro, Haruhara Kotaro, Sasaki Takaya, Ueda Hiroyuki, Joh Kensuke, Tsuboi Nobuo, Yokoo Takashi

机构信息

Division of Nephrology and Hypertension, Department of Internal Medicine, Jikei University School of Medicine, 3-25-8 Nishi-Shimbashi, Minato-Ku, Tokyo, 105-8461, Japan.

Division of Nephrology, Department of Internal Medicine, Kawaguchi Municipal Medical Center, Saitama, Japan.

出版信息

CEN Case Rep. 2025 Aug 27. doi: 10.1007/s13730-025-01028-8.

Abstract

A 67-year-old man with a history of hypertension and dyslipidemia presented with edema and heavy proteinuria. Light microscopic analysis of kidney biopsy revealed a diffuse segmental membranous feature. Immunofluorescence stain was segmentally positive for IgA, galactose-deficient IgA1, both κ and λ light chains, and C3 along the glomerular capillary walls, but negative for IgG, IgM, or C1q. Electron microscopy showed subepithelial and intramembranous electron-dense deposits (EDD) in the segmental glomerular capillary walls, along with foot process effacement in the corresponding areas. No EDD was observed in the mesangial or para-mesangial areas. The patient's histopathology revealed membranous nephropathy with a solitary IgA deposition. No clinical findings suggested a secondary cause of membranous nephropathy. Combination therapy with corticosteroids and cyclosporine resulted in proteinuria remission. To our knowledge, this is the first reported case of membranous nephropathy with a segmental pattern associated with solitary IgA and galactose-deficient IgA1 deposition. Further case reports and studies are required to elucidate the pathogenesis of membranous nephropathy, which shows these unique histopathological features.

摘要

一名患有高血压和血脂异常的67岁男性出现水肿和大量蛋白尿。肾脏活检的光镜分析显示弥漫性节段性膜性特征。免疫荧光染色显示沿肾小球毛细血管壁IgA、半乳糖缺乏型IgA1、κ和λ轻链以及C3呈节段性阳性,但IgG、IgM或C1q呈阴性。电子显微镜显示节段性肾小球毛细血管壁上皮下和膜内电子致密沉积物(EDD),相应区域伴有足突消失。在系膜或系膜旁区域未观察到EDD。患者的组织病理学显示为伴有孤立性IgA沉积的膜性肾病。无临床发现提示膜性肾病的继发性病因。皮质类固醇和环孢素联合治疗导致蛋白尿缓解。据我们所知,这是首例报道的具有节段性模式且与孤立性IgA和半乳糖缺乏型IgA1沉积相关的膜性肾病病例。需要进一步的病例报告和研究来阐明具有这些独特组织病理学特征的膜性肾病的发病机制。

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