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一例罕见的磷脂酶A2受体(PLA2R)和血小板反应蛋白基序7A(THSD7A)阳性的特发性膜性肾病。

A rare case of PLA2R- and THSD7A-positive idiopathic membranous nephropathy.

作者信息

Wanderley David Campos, Jones Bárbara Dornelas, Barbosa Fabricio Augusto Marques, Araujo Stanley de Almeida

机构信息

Instituto de Nefropatologia, Belo Horizonte, MG, Brasil.

Universidade Federal de Minas Gerais, Centro de Microscopia Eletrônica, Belo Horizonte, MG, Brasil.

出版信息

J Bras Nefrol. 2019 Oct 28;42(2):254-258. doi: 10.1590/2175-8239-JBN-2019-0077.

Abstract

Idiopathic membranous nephropathy (IMN) is a frequent cause of nephrotic syndrome in adults. In terms of etiology, the condition may be categorized as primary/idiopathic or secondary. Literature on the pathophysiology of IMN has indicated the presence of autoantibodies (PLA2R and THSD7A) directed against podocyte antigens. The detection of antibodies against a domain favors IMN. The presence of autoantibodies against one of the domains would in theory exclude the possibility of there being autoantibodies against the other domain. However, cases of patients with PLA2R- and THSD7A-positive disease have been recently reported, showing that antibodies against two targets may be concomitantly produced via yet unknown pathophysiological mechanisms. This study reports the case of a 46-year-old male patient with nephrotic-range proteinuria, hematuria, hypoalbuminemia, and hypercholesterolemia submitted to biopsy and histopathology examination (LM, IF, IHC, and EM) eventually diagnosed with PLA2R- and THSD7A-positive IMN associated with IgA nephropathy, stressing our experience with the use of IgG subclasses, PLA2R, and THSD7A in the workup for MN and the relevance of adopting a broad and adequate approach to elucidating and acquiring knowledge of the pathophysiology of IMN.

摘要

特发性膜性肾病(IMN)是成人肾病综合征的常见病因。就病因而言,该病可分为原发性/特发性或继发性。关于IMN病理生理学的文献表明存在针对足细胞抗原的自身抗体(PLA2R和THSD7A)。针对某一结构域的抗体检测支持IMN的诊断。理论上,针对其中一个结构域的自身抗体的存在将排除存在针对另一个结构域的自身抗体的可能性。然而,最近有报道称存在PLA2R和THSD7A均阳性的患者病例,表明针对两个靶点的抗体可能通过尚不清楚的病理生理机制同时产生。本研究报告了一例46岁男性患者,其有肾病范围的蛋白尿、血尿、低白蛋白血症和高胆固醇血症,接受了活检和组织病理学检查(光镜、免疫荧光、免疫组化和电镜),最终诊断为与IgA肾病相关的PLA2R和THSD7A均阳性的IMN,强调了我们在MN检查中使用IgG亚类、PLA2R和THSD7A的经验,以及采用广泛而充分的方法来阐明和了解IMN病理生理学的相关性。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6c5b/7427638/c7fe3950c857/2175-8239-jbn-2019-0077-gf01.jpg

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