Mandibular Resorption and Vocal Cord Paralysis: a Catastrophic Form of Systemic Sclerosis
Autor(a) principal: | |
---|---|
Data de Publicação: | 2019 |
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/10400.17/3463 |
Resumo: | Sudden respiratory distress in association with severe weight loss are unusual features of systemic sclerosis (SSc). We report the case of a 56-year-old Caucasian woman with a 9-year history of a diffuse form of SSc who presented with acute stridor due to vocal cord paralysis and required an emergency tracheostomy. She had sought medical attention only after 4 years of disease onset, presenting with a mask-like face, diffuse skin thickening, acro-osteolysis and severe interstitial lung disease. Even though skin tightness improved after immunosuppressive treatment, several spontaneous facial fractures and episodes of dysphagia and choking occurred in the years that followed. At the time of stridor, she was severely malnourished and a percutaneous endoscopic gastrostomy was required for feeding. Permanent vocal cord damage in combination with severe loco-regional bone resorption resulted in severe disability and impaired nutrition. We hereby highlight the features of SSc for which therapy remains challenging. |
id |
RCAP_1b17508d74f1901d765bae5969ac46d1 |
---|---|
oai_identifier_str |
oai:repositorio.chlc.min-saude.pt:10400.17/3463 |
network_acronym_str |
RCAP |
network_name_str |
Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) |
repository_id_str |
7160 |
spelling |
Mandibular Resorption and Vocal Cord Paralysis: a Catastrophic Form of Systemic SclerosisAntibodies, Monoclonal, HumanizedBone ResorptionCalciumDiagnosis, DifferentialDietary SupplementsFemaleHumansMalnutritionMandibleMiddle AgedScleroderma, SystemicTomography, X-Ray ComputedTracheostomyVitamin DVocal Cord ParalysisHCC DAUTOIMHCC IMASudden respiratory distress in association with severe weight loss are unusual features of systemic sclerosis (SSc). We report the case of a 56-year-old Caucasian woman with a 9-year history of a diffuse form of SSc who presented with acute stridor due to vocal cord paralysis and required an emergency tracheostomy. She had sought medical attention only after 4 years of disease onset, presenting with a mask-like face, diffuse skin thickening, acro-osteolysis and severe interstitial lung disease. Even though skin tightness improved after immunosuppressive treatment, several spontaneous facial fractures and episodes of dysphagia and choking occurred in the years that followed. At the time of stridor, she was severely malnourished and a percutaneous endoscopic gastrostomy was required for feeding. Permanent vocal cord damage in combination with severe loco-regional bone resorption resulted in severe disability and impaired nutrition. We hereby highlight the features of SSc for which therapy remains challenging.BMJ Publishing GroupRepositório do Centro Hospitalar Universitário de Lisboa Central, EPEMendonça, PTaulaigo, AVCaetano, AMoraes-Fontes, MF2020-05-22T11:45:01Z2019-02-282019-02-28T00:00:00Zinfo:eu-repo/semantics/publishedVersioninfo:eu-repo/semantics/articleapplication/pdfhttp://hdl.handle.net/10400.17/3463engBMJ Case Rep . 2019 Feb 28;12(2):e22826210.1136/bcr-2018-228262info:eu-repo/semantics/openAccessreponame:Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos)instname:Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informaçãoinstacron:RCAAP2023-03-10T09:43:08Zoai:repositorio.chlc.min-saude.pt:10400.17/3463Portal AgregadorONGhttps://www.rcaap.pt/oai/openaireopendoar:71602024-03-19T17:20:46.479931Repositório 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 |
Mandibular Resorption and Vocal Cord Paralysis: a Catastrophic Form of Systemic Sclerosis |
title |
Mandibular Resorption and Vocal Cord Paralysis: a Catastrophic Form of Systemic Sclerosis |
spellingShingle |
Mandibular Resorption and Vocal Cord Paralysis: a Catastrophic Form of Systemic Sclerosis Mendonça, P Antibodies, Monoclonal, Humanized Bone Resorption Calcium Diagnosis, Differential Dietary Supplements Female Humans Malnutrition Mandible Middle Aged Scleroderma, Systemic Tomography, X-Ray Computed Tracheostomy Vitamin D Vocal Cord Paralysis HCC DAUTOIM HCC IMA |
title_short |
Mandibular Resorption and Vocal Cord Paralysis: a Catastrophic Form of Systemic Sclerosis |
title_full |
Mandibular Resorption and Vocal Cord Paralysis: a Catastrophic Form of Systemic Sclerosis |
title_fullStr |
Mandibular Resorption and Vocal Cord Paralysis: a Catastrophic Form of Systemic Sclerosis |
title_full_unstemmed |
Mandibular Resorption and Vocal Cord Paralysis: a Catastrophic Form of Systemic Sclerosis |
title_sort |
Mandibular Resorption and Vocal Cord Paralysis: a Catastrophic Form of Systemic Sclerosis |
author |
Mendonça, P |
author_facet |
Mendonça, P Taulaigo, AV Caetano, A Moraes-Fontes, MF |
author_role |
author |
author2 |
Taulaigo, AV Caetano, A Moraes-Fontes, MF |
author2_role |
author author author |
dc.contributor.none.fl_str_mv |
Repositório do Centro Hospitalar Universitário de Lisboa Central, EPE |
dc.contributor.author.fl_str_mv |
Mendonça, P Taulaigo, AV Caetano, A Moraes-Fontes, MF |
dc.subject.por.fl_str_mv |
Antibodies, Monoclonal, Humanized Bone Resorption Calcium Diagnosis, Differential Dietary Supplements Female Humans Malnutrition Mandible Middle Aged Scleroderma, Systemic Tomography, X-Ray Computed Tracheostomy Vitamin D Vocal Cord Paralysis HCC DAUTOIM HCC IMA |
topic |
Antibodies, Monoclonal, Humanized Bone Resorption Calcium Diagnosis, Differential Dietary Supplements Female Humans Malnutrition Mandible Middle Aged Scleroderma, Systemic Tomography, X-Ray Computed Tracheostomy Vitamin D Vocal Cord Paralysis HCC DAUTOIM HCC IMA |
description |
Sudden respiratory distress in association with severe weight loss are unusual features of systemic sclerosis (SSc). We report the case of a 56-year-old Caucasian woman with a 9-year history of a diffuse form of SSc who presented with acute stridor due to vocal cord paralysis and required an emergency tracheostomy. She had sought medical attention only after 4 years of disease onset, presenting with a mask-like face, diffuse skin thickening, acro-osteolysis and severe interstitial lung disease. Even though skin tightness improved after immunosuppressive treatment, several spontaneous facial fractures and episodes of dysphagia and choking occurred in the years that followed. At the time of stridor, she was severely malnourished and a percutaneous endoscopic gastrostomy was required for feeding. Permanent vocal cord damage in combination with severe loco-regional bone resorption resulted in severe disability and impaired nutrition. We hereby highlight the features of SSc for which therapy remains challenging. |
publishDate |
2019 |
dc.date.none.fl_str_mv |
2019-02-28 2019-02-28T00:00:00Z 2020-05-22T11:45:01Z |
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/10400.17/3463 |
url |
http://hdl.handle.net/10400.17/3463 |
dc.language.iso.fl_str_mv |
eng |
language |
eng |
dc.relation.none.fl_str_mv |
BMJ Case Rep . 2019 Feb 28;12(2):e228262 10.1136/bcr-2018-228262 |
dc.rights.driver.fl_str_mv |
info:eu-repo/semantics/openAccess |
eu_rights_str_mv |
openAccess |
dc.format.none.fl_str_mv |
application/pdf |
dc.publisher.none.fl_str_mv |
BMJ Publishing Group |
publisher.none.fl_str_mv |
BMJ Publishing Group |
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) |
collection |
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 |
repository.mail.fl_str_mv |
|
_version_ |
1799131303789985792 |