Particularly severe form of refractory gastrointestinal involvement in systemic sclerosis
Autor(a) principal: | |
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Data de Publicação: | 2023 |
Outros Autores: | , , |
Tipo de documento: | Artigo |
Idioma: | eng |
Título da fonte: | Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) |
Texto Completo: | http://hdl.handle.net/10400.21/15925 |
Resumo: | A woman with systemic sclerosis presents with a severe and rapidly progressive form of gastrointestinal involvement, mainly marked by recurrent refractory episodes of pseudo-obstruction, culminating in severe malnutrition and dependence on parenteral nutrition. The impact on her quality of life was extremely significant. As a last resort, she started intravenous immunoglobulin with progressive improvement of her symptoms, allowing for the reinstitution of oral diet and removal of parenteral nutrition. After more than 1 year, she maintains clinical stability. Systemic sclerosis has a heterogeneous phenotype, but gastrointestinal involvement is one of the most frequent. Severe manifestations are rare but can lead to severe malnutrition and are associated with high morbidity and mortality rates. Their management is challenging, as the available treatments are still very limited. A better understanding of its pathophysiology, which seems to be unique, is essential to provide more effective treatments and improve quality of life. |
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Particularly severe form of refractory gastrointestinal involvement in systemic sclerosisConnective tissue diseaseMalabsorptionMalnutritionParenteral feedingEnteral feedingQuality of lifeA woman with systemic sclerosis presents with a severe and rapidly progressive form of gastrointestinal involvement, mainly marked by recurrent refractory episodes of pseudo-obstruction, culminating in severe malnutrition and dependence on parenteral nutrition. The impact on her quality of life was extremely significant. As a last resort, she started intravenous immunoglobulin with progressive improvement of her symptoms, allowing for the reinstitution of oral diet and removal of parenteral nutrition. After more than 1 year, she maintains clinical stability. Systemic sclerosis has a heterogeneous phenotype, but gastrointestinal involvement is one of the most frequent. Severe manifestations are rare but can lead to severe malnutrition and are associated with high morbidity and mortality rates. Their management is challenging, as the available treatments are still very limited. A better understanding of its pathophysiology, which seems to be unique, is essential to provide more effective treatments and improve quality of life.BMJRCIPLSilva, Ana Bento daLourenço, Maria HelenaCunha-Branco, JaimeGonçalves, Maria João2023-042023-04-01T00:00:00Z2025-04-21T00:00:00Zinfo:eu-repo/semantics/publishedVersioninfo:eu-repo/semantics/articleapplication/pdfhttp://hdl.handle.net/10400.21/15925engSilva AB, Lourenço MH, Cunha-Branco J, Gonçalves MJ. Particularly severe form of refractory gastrointestinal involvement in systemic sclerosis. BMJ Case Rep. 2023;16(4):e254537.10.1136/bcr-2022-254537info:eu-repo/semantics/embargoedAccessreponame:Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos)instname:Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informaçãoinstacron:RCAAP2023-08-03T10:14:01Zoai:repositorio.ipl.pt:10400.21/15925Portal AgregadorONGhttps://www.rcaap.pt/oai/openaireopendoar:71602024-03-19T20:23:30.732600Repositório 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 |
Particularly severe form of refractory gastrointestinal involvement in systemic sclerosis |
title |
Particularly severe form of refractory gastrointestinal involvement in systemic sclerosis |
spellingShingle |
Particularly severe form of refractory gastrointestinal involvement in systemic sclerosis Silva, Ana Bento da Connective tissue disease Malabsorption Malnutrition Parenteral feeding Enteral feeding Quality of life |
title_short |
Particularly severe form of refractory gastrointestinal involvement in systemic sclerosis |
title_full |
Particularly severe form of refractory gastrointestinal involvement in systemic sclerosis |
title_fullStr |
Particularly severe form of refractory gastrointestinal involvement in systemic sclerosis |
title_full_unstemmed |
Particularly severe form of refractory gastrointestinal involvement in systemic sclerosis |
title_sort |
Particularly severe form of refractory gastrointestinal involvement in systemic sclerosis |
author |
Silva, Ana Bento da |
author_facet |
Silva, Ana Bento da Lourenço, Maria Helena Cunha-Branco, Jaime Gonçalves, Maria João |
author_role |
author |
author2 |
Lourenço, Maria Helena Cunha-Branco, Jaime Gonçalves, Maria João |
author2_role |
author author author |
dc.contributor.none.fl_str_mv |
RCIPL |
dc.contributor.author.fl_str_mv |
Silva, Ana Bento da Lourenço, Maria Helena Cunha-Branco, Jaime Gonçalves, Maria João |
dc.subject.por.fl_str_mv |
Connective tissue disease Malabsorption Malnutrition Parenteral feeding Enteral feeding Quality of life |
topic |
Connective tissue disease Malabsorption Malnutrition Parenteral feeding Enteral feeding Quality of life |
description |
A woman with systemic sclerosis presents with a severe and rapidly progressive form of gastrointestinal involvement, mainly marked by recurrent refractory episodes of pseudo-obstruction, culminating in severe malnutrition and dependence on parenteral nutrition. The impact on her quality of life was extremely significant. As a last resort, she started intravenous immunoglobulin with progressive improvement of her symptoms, allowing for the reinstitution of oral diet and removal of parenteral nutrition. After more than 1 year, she maintains clinical stability. Systemic sclerosis has a heterogeneous phenotype, but gastrointestinal involvement is one of the most frequent. Severe manifestations are rare but can lead to severe malnutrition and are associated with high morbidity and mortality rates. Their management is challenging, as the available treatments are still very limited. A better understanding of its pathophysiology, which seems to be unique, is essential to provide more effective treatments and improve quality of life. |
publishDate |
2023 |
dc.date.none.fl_str_mv |
2023-04 2023-04-01T00:00:00Z 2025-04-21T00:00:00Z |
dc.type.status.fl_str_mv |
info:eu-repo/semantics/publishedVersion |
dc.type.driver.fl_str_mv |
info:eu-repo/semantics/article |
format |
article |
status_str |
publishedVersion |
dc.identifier.uri.fl_str_mv |
http://hdl.handle.net/10400.21/15925 |
url |
http://hdl.handle.net/10400.21/15925 |
dc.language.iso.fl_str_mv |
eng |
language |
eng |
dc.relation.none.fl_str_mv |
Silva AB, Lourenço MH, Cunha-Branco J, Gonçalves MJ. Particularly severe form of refractory gastrointestinal involvement in systemic sclerosis. BMJ Case Rep. 2023;16(4):e254537. 10.1136/bcr-2022-254537 |
dc.rights.driver.fl_str_mv |
info:eu-repo/semantics/embargoedAccess |
eu_rights_str_mv |
embargoedAccess |
dc.format.none.fl_str_mv |
application/pdf |
dc.publisher.none.fl_str_mv |
BMJ |
publisher.none.fl_str_mv |
BMJ |
dc.source.none.fl_str_mv |
reponame:Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) instname:Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informação instacron:RCAAP |
instname_str |
Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informação |
instacron_str |
RCAAP |
institution |
RCAAP |
reponame_str |
Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) |
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Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) |
repository.name.fl_str_mv |
Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) - Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informação |
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1817551209415835648 |