An Interesting Case of Nonlupus Full-House Nephropathy.

Case Reports in Nephrology Pub Date : 2021-12-28 eCollection Date: 2021-01-01 DOI:10.1155/2021/9043003
Satyanand Sathi, Alok Sharma, Anil Kumar Garg, Virendra Singh Saini, Manoj Kumar Singh, Devinder Vohra, Arvind Trivedi
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引用次数: 1

Abstract

Full-house immunofluorescence and endothelial tubuloreticular inclusions are known as characteristic features of lupus nephritis. However, both features are not pathognomonic for lupus nephritis. A kidney biopsy specimen showing full-house immunofluorescence pattern in the absence of autoantibodies and classical clinical features of Systemic Lupus Erythematosus (SLE) is now considered as nonlupus full-house nephropathy (FHN). Nonlupus FHN may be idiopathic or due to other disease processes known as secondary nonlupus FHN. Here, we report the case of a 36-year-old female who presented with nephrotic proteinuria with bland urine sediment. Additional analyses revealed normal serum antinuclear antibody (ANA), normal anti-double-stranded DNA (anti-dsDNA) antibodies, and normal serum C3 and C4 levels. A renal biopsy showed a normal-appearing glomerulus without any proliferation or capillary wall thickening and widespread glomerular immune deposits (full-house effect; IgA, IgG, IgM, C3, and C1Q) on direct immunofluorescence. Renal electron microscopy showed diffuse effacement of visceral epithelial cell foot processes and mesangial electron dense deposits. The patient was diagnosed as nonlupus FHN. There is a controversial role of steroids and other immunosuppressive drugs in the treatment of nonlupus FHN patients, but our case patient responded favourably to steroid therapy. The term nonlupus FHN can be used as an umbrella term for patients who do not satisfy the clinical and serological criteria of SLE.

Abstract Image

Abstract Image

一例有趣的非狼疮性全屋肾病。
全身免疫荧光和内皮管网状包涵体是狼疮肾炎的特征性特征。然而,这两个特征都不是狼疮性肾炎的典型症状。肾活检标本在没有自身抗体和系统性红斑狼疮(SLE)的典型临床特征的情况下显示全屋免疫荧光模式,现在被认为是非狼疮性全屋肾病(FHN)。非狼疮性FHN可能是特发性的或由于其他疾病过程称为继发性非狼疮性FHN。在这里,我们报告一个36岁的女性谁提出了肾病蛋白尿淡性尿沉淀。进一步分析显示血清抗核抗体(ANA)、抗双链DNA(抗dsdna)抗体正常,血清C3和C4水平正常。肾活检显示肾小球外观正常,未见增生或毛细血管壁增厚,肾小球免疫沉积广泛存在(全室效应;IgA, IgG, IgM, C3和C1Q)的直接免疫荧光。肾脏电镜显示内脏上皮细胞足突弥漫性消失及系膜电子致密沉积。患者被诊断为非狼疮性FHN。类固醇和其他免疫抑制药物在治疗非狼疮性FHN患者中的作用存在争议,但本病例患者对类固醇治疗反应良好。术语非狼疮性FHN可以作为不满足SLE临床和血清学标准的患者的总称。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
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来源期刊
Case Reports in Nephrology
Case Reports in Nephrology Medicine-Nephrology
CiteScore
1.70
自引率
0.00%
发文量
32
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