Immunoglobulin G4-related disease mimicking multiple myeloma
Main Author: | |
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Publication Date: | 2018 |
Other Authors: | , , , , , |
Format: | Report |
Language: | eng |
Source: | Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) |
Download full: | http://scielo.pt/scielo.php?script=sci_arttext&pid=S0872-01692018000300008 |
Summary: | Immunoglobulin G4-related disease (IgG4-RD) is a rare, poorly understood immune mediated disorder. It is characterized by a wide clinical spectrum depending on the organs affected. Serum IgG4 may be elevated, but this is not mandatory. Imaging abnormalities are usually detected in the affected organs, which typically show enlarged dimensions. Definitive diagnosis is made upon tissue biopsy demonstrating lymphoplasmacytic infiltration with predominance of polyclonal IgG4-positive plasma cells, storiform fibrosis and obliterative phlebitis. The most common renal manifestations [(IgG4-related kidney disease (IgG4#8209;RKD)] include tubulointerstitial nephritis, membranous glomerulonephritis and pyelitis. There is, usually, good therapeutic response to corticosteroids, but rituximab may be needed in cases of relapsing or resistant disease. A diagnostic challenge, and because it diagnosis needs specific immunohistochemical staining techniques, IgG4-RKD should be contemplated in the differential diagnosis of obscure kidney disease in order not to be missed. |
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Immunoglobulin G4-related disease mimicking multiple myelomaImmunoglobulin G4immunoglobulin G4-related diseaseimmunoglobulin G4-related kidney diseaselymphoplasmacytic infiltrationstoriform fibrosistubulointerstitial nephritisImmunoglobulin G4-related disease (IgG4-RD) is a rare, poorly understood immune mediated disorder. It is characterized by a wide clinical spectrum depending on the organs affected. Serum IgG4 may be elevated, but this is not mandatory. Imaging abnormalities are usually detected in the affected organs, which typically show enlarged dimensions. Definitive diagnosis is made upon tissue biopsy demonstrating lymphoplasmacytic infiltration with predominance of polyclonal IgG4-positive plasma cells, storiform fibrosis and obliterative phlebitis. The most common renal manifestations [(IgG4-related kidney disease (IgG4#8209;RKD)] include tubulointerstitial nephritis, membranous glomerulonephritis and pyelitis. There is, usually, good therapeutic response to corticosteroids, but rituximab may be needed in cases of relapsing or resistant disease. A diagnostic challenge, and because it diagnosis needs specific immunohistochemical staining techniques, IgG4-RKD should be contemplated in the differential diagnosis of obscure kidney disease in order not to be missed.Sociedade Portuguesa de Nefrologia2018-09-01info:eu-repo/semantics/publishedVersioninfo:eu-repo/semantics/reporttext/htmlhttp://scielo.pt/scielo.php?script=sci_arttext&pid=S0872-01692018000300008Portuguese Journal of Nephrology & Hypertension v.32 n.3 2018reponame:Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos)instname:Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informaçãoinstacron:RCAAPenghttp://scielo.pt/scielo.php?script=sci_arttext&pid=S0872-01692018000300008Costa,Marta SofiaSilva,AndreiaCosta,LuísaRodrigues,AnaBarra,TiagoLemos,SérgioGarrido,Jesúsinfo:eu-repo/semantics/openAccess2024-02-06T17:04:59Zoai:scielo:S0872-01692018000300008Portal AgregadorONGhttps://www.rcaap.pt/oai/openaireopendoar:71602024-03-20T02:19:01.003673Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) - Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informaçãofalse |
dc.title.none.fl_str_mv |
Immunoglobulin G4-related disease mimicking multiple myeloma |
title |
Immunoglobulin G4-related disease mimicking multiple myeloma |
spellingShingle |
Immunoglobulin G4-related disease mimicking multiple myeloma Costa,Marta Sofia Immunoglobulin G4 immunoglobulin G4-related disease immunoglobulin G4-related kidney disease lymphoplasmacytic infiltration storiform fibrosis tubulointerstitial nephritis |
title_short |
Immunoglobulin G4-related disease mimicking multiple myeloma |
title_full |
Immunoglobulin G4-related disease mimicking multiple myeloma |
title_fullStr |
Immunoglobulin G4-related disease mimicking multiple myeloma |
title_full_unstemmed |
Immunoglobulin G4-related disease mimicking multiple myeloma |
title_sort |
Immunoglobulin G4-related disease mimicking multiple myeloma |
author |
Costa,Marta Sofia |
author_facet |
Costa,Marta Sofia Silva,Andreia Costa,Luísa Rodrigues,Ana Barra,Tiago Lemos,Sérgio Garrido,Jesús |
author_role |
author |
author2 |
Silva,Andreia Costa,Luísa Rodrigues,Ana Barra,Tiago Lemos,Sérgio Garrido,Jesús |
author2_role |
author author author author author author |
dc.contributor.author.fl_str_mv |
Costa,Marta Sofia Silva,Andreia Costa,Luísa Rodrigues,Ana Barra,Tiago Lemos,Sérgio Garrido,Jesús |
dc.subject.por.fl_str_mv |
Immunoglobulin G4 immunoglobulin G4-related disease immunoglobulin G4-related kidney disease lymphoplasmacytic infiltration storiform fibrosis tubulointerstitial nephritis |
topic |
Immunoglobulin G4 immunoglobulin G4-related disease immunoglobulin G4-related kidney disease lymphoplasmacytic infiltration storiform fibrosis tubulointerstitial nephritis |
description |
Immunoglobulin G4-related disease (IgG4-RD) is a rare, poorly understood immune mediated disorder. It is characterized by a wide clinical spectrum depending on the organs affected. Serum IgG4 may be elevated, but this is not mandatory. Imaging abnormalities are usually detected in the affected organs, which typically show enlarged dimensions. Definitive diagnosis is made upon tissue biopsy demonstrating lymphoplasmacytic infiltration with predominance of polyclonal IgG4-positive plasma cells, storiform fibrosis and obliterative phlebitis. The most common renal manifestations [(IgG4-related kidney disease (IgG4#8209;RKD)] include tubulointerstitial nephritis, membranous glomerulonephritis and pyelitis. There is, usually, good therapeutic response to corticosteroids, but rituximab may be needed in cases of relapsing or resistant disease. A diagnostic challenge, and because it diagnosis needs specific immunohistochemical staining techniques, IgG4-RKD should be contemplated in the differential diagnosis of obscure kidney disease in order not to be missed. |
publishDate |
2018 |
dc.date.none.fl_str_mv |
2018-09-01 |
dc.type.status.fl_str_mv |
info:eu-repo/semantics/publishedVersion |
dc.type.driver.fl_str_mv |
info:eu-repo/semantics/report |
format |
report |
status_str |
publishedVersion |
dc.identifier.uri.fl_str_mv |
http://scielo.pt/scielo.php?script=sci_arttext&pid=S0872-01692018000300008 |
url |
http://scielo.pt/scielo.php?script=sci_arttext&pid=S0872-01692018000300008 |
dc.language.iso.fl_str_mv |
eng |
language |
eng |
dc.relation.none.fl_str_mv |
http://scielo.pt/scielo.php?script=sci_arttext&pid=S0872-01692018000300008 |
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 Portuguesa de Nefrologia |
publisher.none.fl_str_mv |
Sociedade Portuguesa de Nefrologia |
dc.source.none.fl_str_mv |
Portuguese Journal of Nephrology & Hypertension v.32 n.3 2018 reponame:Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) instname:Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informação instacron:RCAAP |
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Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informação |
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RCAAP |
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RCAAP |
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Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) |
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Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) |
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Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) - Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informação |
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1799137280122683392 |