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抗双链DNA阴性且抗Ro阳性的狼疮性肾炎:1例罕见病例报告

Anti-dsDNA negative and anti-Ro positive lupus nephritis: a report of a rare case.

作者信息

Jain D, Aggarwal H K, Kaverappa V, Dhayia S, Jain P, Yadav S

机构信息

Department of Medicine, Pt. B.D. Sharma University of Health Sciences.

出版信息

Reumatismo. 2014 Mar 17;65(6):302-6. doi: 10.4081/reumatismo.2013.706.

Abstract

Systemic lupus erythematosus (SLE) is a multisystem autoimmune disease, characterized by an autoantibody response to various nuclear and cytoplasmic antigens. Renal disease in SLE occurs in 40-75% of patients, most often within five years of onset of disease, and is one of the strongest predictors of a poor outcome. A hallmark of glomerular involvement in lupus nephritis is the presence of autoantibodies against double-stranded DNA (dsDNA). Its level usually correlates with disease activity. Our patient presented with a rash resembling malar rash and features of nephrotic syndrome. On investigating, patient was found to have pancytopenia, raised erythrocyte sedimentation rate and depressed serum C3 levels with positivity of antinuclear antibodies and anti- Ro antibodies. However, most of the markers of lupus nephritis including anti dsDNA antibody were negative. Renal biopsy showed features of lupus nephritis (class-IV). Differential item functioning studies showed a full house immunoflourescence staining pattern characteristic of lupus nephritis. Association of Anti-Ro antibody alone with lupus nephritis is less known in literature. Negativity of anti-dsDNA antibody, which is usually considered to be diagnostic of lupus nephritis, poses a diagnostic dilemma short of renal biopsy. Till date only very few cases of non-drug induced lupus nephritis with negative dsDNA antibodies have been reported. In this report we wish to highlight a case of lupus nephritis which was negative for its specific anti dsDNA antibodies and with possible role of anti-Ro antibodies in the pathogenesis of lupus nephritis although the underlying mechanism is incompletely understood.

摘要

系统性红斑狼疮(SLE)是一种多系统自身免疫性疾病,其特征是针对各种核抗原和胞质抗原产生自身抗体反应。SLE患者中40%-75%会出现肾脏疾病,最常发生在疾病发病后的五年内,并且是预后不良的最强预测指标之一。狼疮性肾炎肾小球受累的一个标志是存在抗双链DNA(dsDNA)自身抗体。其水平通常与疾病活动度相关。我们的患者表现出类似蝶形红斑的皮疹和肾病综合征的特征。经检查,发现患者全血细胞减少、红细胞沉降率升高、血清C3水平降低,抗核抗体和抗Ro抗体呈阳性。然而,包括抗dsDNA抗体在内的大多数狼疮性肾炎标志物均为阴性。肾活检显示狼疮性肾炎(IV类)的特征。差异项目功能研究显示了狼疮性肾炎特有的满堂免疫荧光染色模式。单独抗Ro抗体与狼疮性肾炎的关联在文献中鲜为人知。通常被认为可诊断狼疮性肾炎的抗dsDNA抗体阴性,在缺乏肾活检的情况下构成了诊断难题。迄今为止,仅有极少数非药物性诱导的狼疮性肾炎且dsDNA抗体阴性的病例被报道。在本报告中,我们希望强调一例狼疮性肾炎病例,其特异性抗dsDNA抗体为阴性,尽管潜在机制尚不完全清楚,但抗Ro抗体可能在狼疮性肾炎的发病机制中发挥作用。

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