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IgG4-Related Disease: A Multispecialty Condition
IgG4-related disease (IgG4-RD) is a recently recognized group of conditions, characterized by tumor-like swelling of involved organs, lymphoplasmacytic infiltrate rich in IgG4-positive plasma cells, variable degrees of fibrosis, and elevated serum IgG4 concentrations. Currently IgG4-RD is recognized...
Autores principales: | , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Hindawi Publishing Corporation
2014
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4260427/ https://www.ncbi.nlm.nih.gov/pubmed/25506457 http://dx.doi.org/10.1155/2014/723493 |
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author | Usêda Santana, Iuri da Fonseca, Emanuela Pimenta Santiago, Mittermayer Barreto |
author_facet | Usêda Santana, Iuri da Fonseca, Emanuela Pimenta Santiago, Mittermayer Barreto |
author_sort | Usêda Santana, Iuri |
collection | PubMed |
description | IgG4-related disease (IgG4-RD) is a recently recognized group of conditions, characterized by tumor-like swelling of involved organs, lymphoplasmacytic infiltrate rich in IgG4-positive plasma cells, variable degrees of fibrosis, and elevated serum IgG4 concentrations. Currently IgG4-RD is recognized as a systemic condition that can affect several organs and tissues. Herein we report the case of a 34-year-old male patient who was admitted to our hospital with diffuse abdominal pain, weight loss, and painful stiffness in his neck. He had a history of tumoral mass of the left maxillary region, right palpebral ptosis with protrusion of the eyeball, and chronic dry cough for about 6 years. Laboratory tests revealed polyclonal hypergammaglobulinemia and increased serum IgG4 levels. Immunohistochemical staining of the maxillary biopsy was compatible with IgG4-RD. He had an excellent response to corticosteroid therapy. This case highlights that IgG4-RD should be included in the differential diagnosis with multisystem diseases. |
format | Online Article Text |
id | pubmed-4260427 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2014 |
publisher | Hindawi Publishing Corporation |
record_format | MEDLINE/PubMed |
spelling | pubmed-42604272014-12-14 IgG4-Related Disease: A Multispecialty Condition Usêda Santana, Iuri da Fonseca, Emanuela Pimenta Santiago, Mittermayer Barreto Case Rep Rheumatol Case Report IgG4-related disease (IgG4-RD) is a recently recognized group of conditions, characterized by tumor-like swelling of involved organs, lymphoplasmacytic infiltrate rich in IgG4-positive plasma cells, variable degrees of fibrosis, and elevated serum IgG4 concentrations. Currently IgG4-RD is recognized as a systemic condition that can affect several organs and tissues. Herein we report the case of a 34-year-old male patient who was admitted to our hospital with diffuse abdominal pain, weight loss, and painful stiffness in his neck. He had a history of tumoral mass of the left maxillary region, right palpebral ptosis with protrusion of the eyeball, and chronic dry cough for about 6 years. Laboratory tests revealed polyclonal hypergammaglobulinemia and increased serum IgG4 levels. Immunohistochemical staining of the maxillary biopsy was compatible with IgG4-RD. He had an excellent response to corticosteroid therapy. This case highlights that IgG4-RD should be included in the differential diagnosis with multisystem diseases. Hindawi Publishing Corporation 2014 2014-11-24 /pmc/articles/PMC4260427/ /pubmed/25506457 http://dx.doi.org/10.1155/2014/723493 Text en Copyright © 2014 Iuri Usêda Santana et al. https://creativecommons.org/licenses/by/3.0/ This is an open access article distributed under 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 Usêda Santana, Iuri da Fonseca, Emanuela Pimenta Santiago, Mittermayer Barreto IgG4-Related Disease: A Multispecialty Condition |
title | IgG4-Related Disease: A Multispecialty Condition |
title_full | IgG4-Related Disease: A Multispecialty Condition |
title_fullStr | IgG4-Related Disease: A Multispecialty Condition |
title_full_unstemmed | IgG4-Related Disease: A Multispecialty Condition |
title_short | IgG4-Related Disease: A Multispecialty Condition |
title_sort | igg4-related disease: a multispecialty condition |
topic | Case Report |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4260427/ https://www.ncbi.nlm.nih.gov/pubmed/25506457 http://dx.doi.org/10.1155/2014/723493 |
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