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1例干燥综合征合并膜性肾病

[A case of Sjögren's syndrome complicated by membranous nephropathy].

作者信息

Kosugi E, Kikuchi Y, Shiina N, Tojo Y, Nagai Y, Kameyama M, Inokuchi T, Kawamura S, Isogai S

机构信息

Second Department of Internal Medicine, Toho University School of Medicine, Tokyo, Japan.

出版信息

Nihon Jinzo Gakkai Shi. 1996 Sep;38(9):413-6.

PMID:8913094
Abstract

A case of Sjögren's syndrome (SjS) complicated by membranous nephropathy (MN) is presented. A 50-year-old female was admitted to Toho University Hospital because of overt proteinuria (5g/day). She had xerotic keratitis in addition to a renal disorder, and laboratory data showed positive anti-nuclear antibody (ANA) and anti-SSA antibody. The specimens from renal biopsy revealed mild thickening of the glomerular basement membrane under light microscopy, positive IgG along the capillary walls revealed by immunofluorescence, and sparse and irregular subepithelial electron dense deposits seen under electron microscopy. No interstitial changes were observed. From these findings, she was diagnosed as having SjS complicated by MN. Proteinuria gradually decreased with a reduction in serum levels of ANA and anti-SSA antibody following corticosteroid therapy. Although renal interstitial lesions occasionally develop in patients with SjS, glomerular changes, especially MN, are very rare. We suspect that immunocomplexes, such as anti-SSA antibody, revealed in SjS patients could be responsible for the glomerular lesions, leading to MN.

摘要

本文报告1例合并膜性肾病(MN)的干燥综合征(SjS)病例。一名50岁女性因明显蛋白尿(5g/天)入住东邦大学医院。除肾脏疾病外,她还患有干性角膜炎,实验室检查显示抗核抗体(ANA)和抗SSA抗体呈阳性。肾活检标本在光学显微镜下显示肾小球基底膜轻度增厚,免疫荧光显示沿毛细血管壁IgG呈阳性,电子显微镜下可见稀疏且不规则的上皮下电子致密沉积物。未观察到间质改变。根据这些发现,她被诊断为SjS合并MN。皮质类固醇治疗后,随着ANA和抗SSA抗体血清水平的降低,蛋白尿逐渐减少。虽然SjS患者偶尔会出现肾间质病变,但肾小球改变,尤其是MN,非常罕见。我们怀疑SjS患者体内出现的免疫复合物,如抗SSA抗体,可能是导致肾小球病变进而引发MN的原因。

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