Erasmus Syndrome: an Underrecognized Entity
Autor(a) principal: | |
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Data de Publicação: | 2022 |
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/10400.17/4464 |
Resumo: | We present a case of a 33-year-old male who worked as a plumber and a locksmith. The patient presented with diffuse myalgia and asthenia, skin sclerosis and puffy fingers, Raynaud's phenomenon, exertional dyspnea and erectile dysfunction. The presence of specific autoantibodies enabled the diagnosis of systemic sclerosis. Chest-computed tomography revealed upper lobe consolidation. After extensive evaluation, the multidisciplinary interstitial lung disease team concluded that the patient also had advanced silicosis. After a year, there was significant clinical, radiologic, and functional deterioration of the lung disease. The patient was referred for lung transplant. Silica inhalation is the cause of silicosis but is also implicated in the development of systemic sclerosis (Erasmus syndrome). Although they share a common risk factor, it is rare to find both diseases co-existing. We present this case of a young patient where both diseases presented aggressively in order to raise awareness to this association. |
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Erasmus Syndrome: an Underrecognized EntitySíndrome de Erasmus: uma Entidade Pouco ReconhecidaHSM PNEUHSAC MEDAdultMaleHumansLungLung Diseases, Interstitial*Scleroderma, Systemic* / diagnosisSilicosis* / complicationsSyndromeSilicosis* / diagnosisWe present a case of a 33-year-old male who worked as a plumber and a locksmith. The patient presented with diffuse myalgia and asthenia, skin sclerosis and puffy fingers, Raynaud's phenomenon, exertional dyspnea and erectile dysfunction. The presence of specific autoantibodies enabled the diagnosis of systemic sclerosis. Chest-computed tomography revealed upper lobe consolidation. After extensive evaluation, the multidisciplinary interstitial lung disease team concluded that the patient also had advanced silicosis. After a year, there was significant clinical, radiologic, and functional deterioration of the lung disease. The patient was referred for lung transplant. Silica inhalation is the cause of silicosis but is also implicated in the development of systemic sclerosis (Erasmus syndrome). Although they share a common risk factor, it is rare to find both diseases co-existing. We present this case of a young patient where both diseases presented aggressively in order to raise awareness to this association.Apresentamos o caso de um homem de 33 anos que trabalhava como canalizador e serralheiro. Apresentava mialgias e astenia, esclerose cutânea e puffy fingers, fenómeno de Raynaud, dispneia de esforço e disfunção erétil. A presença de autoanticorpos específicos permitiu o diagnóstico de esclerose sistémica. A tomografia computadorizada de tórax revelou consolidações dos lobos superiores. Após extensa avaliação, o grupo multidisciplinar de doenças do interstício concluiu que o doente tinha também silicose avançada. Após um ano, houve agravamento clínico, radiológico e funcional significativo da doença pulmonar. O doente foi encaminhado para transplante pulmonar. A inalação de sílica é a causa da silicose, mas também está implicada no desenvolvimento da esclerose sistémica (síndrome de Erasmus). Embora tenham um fator de risco comum, é raro encontrar as duas doenças simultaneamente. Apresentamos o caso de um doente jovem em que ambas as doenças se apresentaram de forma agressiva para alertar sobre esta associação.Ordem dos MédicosRepositório do Centro Hospitalar Universitário de Lisboa Central, EPEMagalhães, AMoreira, IPinheiro, SBorba, A2023-03-17T16:22:10Z2022-022022-02-01T00:00:00Zinfo:eu-repo/semantics/publishedVersioninfo:eu-repo/semantics/articleapplication/pdfhttp://hdl.handle.net/10400.17/4464engActa Med Port . 2023 Feb 1;36(2):122-126.10.20344/amp.16896info: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:RCAAP2023-03-19T05:33:37Zoai:repositorio.chlc.min-saude.pt:10400.17/4464Portal AgregadorONGhttps://www.rcaap.pt/oai/openaireopendoar:71602024-03-19T17:42:55.786657Repositório 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 |
Erasmus Syndrome: an Underrecognized Entity Síndrome de Erasmus: uma Entidade Pouco Reconhecida |
title |
Erasmus Syndrome: an Underrecognized Entity |
spellingShingle |
Erasmus Syndrome: an Underrecognized Entity Magalhães, A HSM PNEU HSAC MED Adult Male Humans Lung Lung Diseases, Interstitial* Scleroderma, Systemic* / diagnosis Silicosis* / complications Syndrome Silicosis* / diagnosis |
title_short |
Erasmus Syndrome: an Underrecognized Entity |
title_full |
Erasmus Syndrome: an Underrecognized Entity |
title_fullStr |
Erasmus Syndrome: an Underrecognized Entity |
title_full_unstemmed |
Erasmus Syndrome: an Underrecognized Entity |
title_sort |
Erasmus Syndrome: an Underrecognized Entity |
author |
Magalhães, A |
author_facet |
Magalhães, A Moreira, I Pinheiro, S Borba, A |
author_role |
author |
author2 |
Moreira, I Pinheiro, S Borba, A |
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 |
Magalhães, A Moreira, I Pinheiro, S Borba, A |
dc.subject.por.fl_str_mv |
HSM PNEU HSAC MED Adult Male Humans Lung Lung Diseases, Interstitial* Scleroderma, Systemic* / diagnosis Silicosis* / complications Syndrome Silicosis* / diagnosis |
topic |
HSM PNEU HSAC MED Adult Male Humans Lung Lung Diseases, Interstitial* Scleroderma, Systemic* / diagnosis Silicosis* / complications Syndrome Silicosis* / diagnosis |
description |
We present a case of a 33-year-old male who worked as a plumber and a locksmith. The patient presented with diffuse myalgia and asthenia, skin sclerosis and puffy fingers, Raynaud's phenomenon, exertional dyspnea and erectile dysfunction. The presence of specific autoantibodies enabled the diagnosis of systemic sclerosis. Chest-computed tomography revealed upper lobe consolidation. After extensive evaluation, the multidisciplinary interstitial lung disease team concluded that the patient also had advanced silicosis. After a year, there was significant clinical, radiologic, and functional deterioration of the lung disease. The patient was referred for lung transplant. Silica inhalation is the cause of silicosis but is also implicated in the development of systemic sclerosis (Erasmus syndrome). Although they share a common risk factor, it is rare to find both diseases co-existing. We present this case of a young patient where both diseases presented aggressively in order to raise awareness to this association. |
publishDate |
2022 |
dc.date.none.fl_str_mv |
2022-02 2022-02-01T00:00:00Z 2023-03-17T16:22:10Z |
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/10400.17/4464 |
url |
http://hdl.handle.net/10400.17/4464 |
dc.language.iso.fl_str_mv |
eng |
language |
eng |
dc.relation.none.fl_str_mv |
Acta Med Port . 2023 Feb 1;36(2):122-126. 10.20344/amp.16896 |
dc.rights.driver.fl_str_mv |
info:eu-repo/semantics/openAccess |
eu_rights_str_mv |
openAccess |
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application/pdf |
dc.publisher.none.fl_str_mv |
Ordem dos Médicos |
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Ordem dos Médicos |
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 |
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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 |
reponame_str |
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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