IgG4-Related Esophageal Disease Presenting as Esophagitis with Chronic Strictures

IgG4-related disease is a recently recognized autoimmune systemic disorder that has been described in various organs. The disease is characterized histologically by a dense lymphoplasmacytic infiltrate with IgG4-positive cells, storiform fibrosis, obliterative phlebitis, and can be associated with s...

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Autores principales: Catarina Correia, Hélder Moreira, Nuno Almeida, Marta Soares, Augusta Cipriano, Pedro Figueiredo
Formato: article
Lenguaje:EN
Publicado: Karger Publishers 2021
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Acceso en línea:https://doaj.org/article/82d319a3719145a8bd360448fd35bfd3
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spelling oai:doaj.org-article:82d319a3719145a8bd360448fd35bfd32021-12-02T12:40:23ZIgG4-Related Esophageal Disease Presenting as Esophagitis with Chronic Strictures2341-45452387-195410.1159/000520271https://doaj.org/article/82d319a3719145a8bd360448fd35bfd32021-11-01T00:00:00Zhttps://www.karger.com/Article/FullText/520271https://doaj.org/toc/2341-4545https://doaj.org/toc/2387-1954IgG4-related disease is a recently recognized autoimmune systemic disorder that has been described in various organs. The disease is characterized histologically by a dense lymphoplasmacytic infiltrate with IgG4-positive cells, storiform fibrosis, obliterative phlebitis, and can be associated with space-occupying lesions. IgG4-related disease involving the upper gastrointestinal tract is rare. We report the case of a 30-year-old female patient with a long-standing history of severe dysphagia and odynophagia. Symptoms persisted despite anti-acid therapy, and control esophagogastroduodenoscopy revealed endoscopic images consistent with a nontransposable stenosis in the proximal esophagus. An underlying autoimmune process was suspected, and topical immunosuppressants were tried to control her disease. The patient maintained disabling dysphagia secondary to chronic esophageal strictures. A diagnosis of probable IgG4-related disease was made after esophageal biopsies. Treatment attempts with topical corticosteroids was not associated with a significant improvement of the symptoms of dysphagia and odynophagia, possibly because of the chronic nature of the disease associated with a high fibrotic component. This report describes a case of IgG4-related esophageal disease presenting as chronic esophagitis with strictures. We also briefly review the main histopathological features and treatment options in IgG4-related disease.Catarina CorreiaHélder MoreiraNuno AlmeidaMarta SoaresAugusta CiprianoPedro FigueiredoKarger Publishersarticleigg4doença relacionada com igg4estenose crónicaDiseases of the digestive system. GastroenterologyRC799-869ENGE: Portuguese Journal of Gastroenterology, Pp 1-5 (2021)
institution DOAJ
collection DOAJ
language EN
topic igg4
doença relacionada com igg4
estenose crónica
Diseases of the digestive system. Gastroenterology
RC799-869
spellingShingle igg4
doença relacionada com igg4
estenose crónica
Diseases of the digestive system. Gastroenterology
RC799-869
Catarina Correia
Hélder Moreira
Nuno Almeida
Marta Soares
Augusta Cipriano
Pedro Figueiredo
IgG4-Related Esophageal Disease Presenting as Esophagitis with Chronic Strictures
description IgG4-related disease is a recently recognized autoimmune systemic disorder that has been described in various organs. The disease is characterized histologically by a dense lymphoplasmacytic infiltrate with IgG4-positive cells, storiform fibrosis, obliterative phlebitis, and can be associated with space-occupying lesions. IgG4-related disease involving the upper gastrointestinal tract is rare. We report the case of a 30-year-old female patient with a long-standing history of severe dysphagia and odynophagia. Symptoms persisted despite anti-acid therapy, and control esophagogastroduodenoscopy revealed endoscopic images consistent with a nontransposable stenosis in the proximal esophagus. An underlying autoimmune process was suspected, and topical immunosuppressants were tried to control her disease. The patient maintained disabling dysphagia secondary to chronic esophageal strictures. A diagnosis of probable IgG4-related disease was made after esophageal biopsies. Treatment attempts with topical corticosteroids was not associated with a significant improvement of the symptoms of dysphagia and odynophagia, possibly because of the chronic nature of the disease associated with a high fibrotic component. This report describes a case of IgG4-related esophageal disease presenting as chronic esophagitis with strictures. We also briefly review the main histopathological features and treatment options in IgG4-related disease.
format article
author Catarina Correia
Hélder Moreira
Nuno Almeida
Marta Soares
Augusta Cipriano
Pedro Figueiredo
author_facet Catarina Correia
Hélder Moreira
Nuno Almeida
Marta Soares
Augusta Cipriano
Pedro Figueiredo
author_sort Catarina Correia
title IgG4-Related Esophageal Disease Presenting as Esophagitis with Chronic Strictures
title_short IgG4-Related Esophageal Disease Presenting as Esophagitis with Chronic Strictures
title_full IgG4-Related Esophageal Disease Presenting as Esophagitis with Chronic Strictures
title_fullStr IgG4-Related Esophageal Disease Presenting as Esophagitis with Chronic Strictures
title_full_unstemmed IgG4-Related Esophageal Disease Presenting as Esophagitis with Chronic Strictures
title_sort igg4-related esophageal disease presenting as esophagitis with chronic strictures
publisher Karger Publishers
publishDate 2021
url https://doaj.org/article/82d319a3719145a8bd360448fd35bfd3
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