A rare case of PLA2R- and THSD7A-positive idiopathic membranous nephropathy
Autor(a) principal: | |
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Data de Publicação: | 2020 |
Outros Autores: | , , |
Tipo de documento: | Artigo |
Idioma: | eng |
Título da fonte: | Jornal Brasileiro de Nefrologia |
Texto Completo: | http://old.scielo.br/scielo.php?script=sci_arttext&pid=S0101-28002020000200254 |
Resumo: | 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. |
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A rare case of PLA2R- and THSD7A-positive idiopathic membranous nephropathyGlomerulonephritis, MembranousReceptors, Phospholipase A2Thrombospondin 1Glomerulonephritis, IGAABSTRACT 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 Nefrologia2020-06-01info:eu-repo/semantics/articleinfo:eu-repo/semantics/publishedVersiontext/htmlhttp://old.scielo.br/scielo.php?script=sci_arttext&pid=S0101-28002020000200254Brazilian Journal of Nephrology v.42 n.2 2020reponame:Jornal Brasileiro de Nefrologiainstname:Sociedade Brasileira de Nefrologia (SBN)instacron:SBN10.1590/2175-8239-jbn-2019-0077info:eu-repo/semantics/openAccessWanderley,David CamposJones,Bárbara DornelasBarbosa,Fabricio Augusto MarquesAraujo,Stanley de Almeidaeng2020-07-09T00:00:00Zoai:scielo:S0101-28002020000200254Revistahttp://www.bjn.org.br/ONGhttps://old.scielo.br/oai/scielo-oai.php||jbn@sbn.org.br2175-82390101-2800opendoar:2020-07-09T00:00Jornal Brasileiro de Nefrologia - Sociedade Brasileira de Nefrologia (SBN)false |
dc.title.none.fl_str_mv |
A rare case of PLA2R- and THSD7A-positive idiopathic membranous nephropathy |
title |
A rare case of PLA2R- and THSD7A-positive idiopathic membranous nephropathy |
spellingShingle |
A rare case of PLA2R- and THSD7A-positive idiopathic membranous nephropathy Wanderley,David Campos Glomerulonephritis, Membranous Receptors, Phospholipase A2 Thrombospondin 1 Glomerulonephritis, IGA |
title_short |
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_sort |
A rare case of PLA2R- and THSD7A-positive idiopathic membranous nephropathy |
author |
Wanderley,David Campos |
author_facet |
Wanderley,David Campos Jones,Bárbara Dornelas Barbosa,Fabricio Augusto Marques Araujo,Stanley de Almeida |
author_role |
author |
author2 |
Jones,Bárbara Dornelas Barbosa,Fabricio Augusto Marques Araujo,Stanley de Almeida |
author2_role |
author author author |
dc.contributor.author.fl_str_mv |
Wanderley,David Campos Jones,Bárbara Dornelas Barbosa,Fabricio Augusto Marques Araujo,Stanley de Almeida |
dc.subject.por.fl_str_mv |
Glomerulonephritis, Membranous Receptors, Phospholipase A2 Thrombospondin 1 Glomerulonephritis, IGA |
topic |
Glomerulonephritis, Membranous Receptors, Phospholipase A2 Thrombospondin 1 Glomerulonephritis, IGA |
description |
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. |
publishDate |
2020 |
dc.date.none.fl_str_mv |
2020-06-01 |
dc.type.driver.fl_str_mv |
info:eu-repo/semantics/article |
dc.type.status.fl_str_mv |
info:eu-repo/semantics/publishedVersion |
format |
article |
status_str |
publishedVersion |
dc.identifier.uri.fl_str_mv |
http://old.scielo.br/scielo.php?script=sci_arttext&pid=S0101-28002020000200254 |
url |
http://old.scielo.br/scielo.php?script=sci_arttext&pid=S0101-28002020000200254 |
dc.language.iso.fl_str_mv |
eng |
language |
eng |
dc.relation.none.fl_str_mv |
10.1590/2175-8239-jbn-2019-0077 |
dc.rights.driver.fl_str_mv |
info:eu-repo/semantics/openAccess |
eu_rights_str_mv |
openAccess |
dc.format.none.fl_str_mv |
text/html |
dc.publisher.none.fl_str_mv |
Sociedade Brasileira de Nefrologia |
publisher.none.fl_str_mv |
Sociedade Brasileira de Nefrologia |
dc.source.none.fl_str_mv |
Brazilian Journal of Nephrology v.42 n.2 2020 reponame:Jornal Brasileiro de Nefrologia instname:Sociedade Brasileira de Nefrologia (SBN) instacron:SBN |
instname_str |
Sociedade Brasileira de Nefrologia (SBN) |
instacron_str |
SBN |
institution |
SBN |
reponame_str |
Jornal Brasileiro de Nefrologia |
collection |
Jornal Brasileiro de Nefrologia |
repository.name.fl_str_mv |
Jornal Brasileiro de Nefrologia - Sociedade Brasileira de Nefrologia (SBN) |
repository.mail.fl_str_mv |
||jbn@sbn.org.br |
_version_ |
1752122066020073472 |