Cargando…
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...
Autores principales: | , , , |
---|---|
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 |
_version_ | 1783570916251271168 |
---|---|
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. |
format | Online Article Text |
id | pubmed-7427638 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2019 |
publisher | Sociedade Brasileira de Nefrologia |
record_format | MEDLINE/PubMed |
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 |
work_keys_str_mv | AT wanderleydavidcampos ararecaseofpla2randthsd7apositiveidiopathicmembranousnephropathy AT jonesbarbaradornelas ararecaseofpla2randthsd7apositiveidiopathicmembranousnephropathy AT barbosafabricioaugustomarques ararecaseofpla2randthsd7apositiveidiopathicmembranousnephropathy AT araujostanleydealmeida ararecaseofpla2randthsd7apositiveidiopathicmembranousnephropathy AT wanderleydavidcampos rarecaseofpla2randthsd7apositiveidiopathicmembranousnephropathy AT jonesbarbaradornelas rarecaseofpla2randthsd7apositiveidiopathicmembranousnephropathy AT barbosafabricioaugustomarques rarecaseofpla2randthsd7apositiveidiopathicmembranousnephropathy AT araujostanleydealmeida rarecaseofpla2randthsd7apositiveidiopathicmembranousnephropathy |