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

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 ag...

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Autores principales: Wanderley, David Campos, Jones, Bárbara Dornelas, Barbosa, Fabricio Augusto Marques, Araujo, Stanley de Almeida
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Sociedade Brasileira de Nefrologia 2019
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7427638/
https://www.ncbi.nlm.nih.gov/pubmed/31663595
http://dx.doi.org/10.1590/2175-8239-JBN-2019-0077
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author Wanderley, David Campos
Jones, Bárbara Dornelas
Barbosa, Fabricio Augusto Marques
Araujo, Stanley de Almeida
author_facet Wanderley, David Campos
Jones, Bárbara Dornelas
Barbosa, Fabricio Augusto Marques
Araujo, Stanley de Almeida
author_sort Wanderley, David Campos
collection PubMed
description 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.
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spelling pubmed-74276382020-08-25 A rare case of PLA2R- and THSD7A-positive idiopathic membranous nephropathy Wanderley, David Campos Jones, Bárbara Dornelas Barbosa, Fabricio Augusto Marques Araujo, Stanley de Almeida J Bras Nefrol Case Report 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. Sociedade Brasileira de Nefrologia 2019-10-28 2020 /pmc/articles/PMC7427638/ /pubmed/31663595 http://dx.doi.org/10.1590/2175-8239-JBN-2019-0077 Text en https://creativecommons.org/licenses/by/4.0/ This is an Open Access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
spellingShingle Case Report
Wanderley, David Campos
Jones, Bárbara Dornelas
Barbosa, Fabricio Augusto Marques
Araujo, Stanley de Almeida
A rare case of PLA2R- and THSD7A-positive idiopathic membranous nephropathy
title A rare case of PLA2R- and THSD7A-positive idiopathic membranous nephropathy
title_full A rare case of PLA2R- and THSD7A-positive idiopathic membranous nephropathy
title_fullStr A rare case of PLA2R- and THSD7A-positive idiopathic membranous nephropathy
title_full_unstemmed A rare case of PLA2R- and THSD7A-positive idiopathic membranous nephropathy
title_short A rare case of PLA2R- and THSD7A-positive idiopathic membranous nephropathy
title_sort rare case of pla2r- and thsd7a-positive idiopathic membranous nephropathy
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7427638/
https://www.ncbi.nlm.nih.gov/pubmed/31663595
http://dx.doi.org/10.1590/2175-8239-JBN-2019-0077
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