Erasmus Syndrome: an Underrecognized Entity

Detalhes bibliográficos
Autor(a) principal: Magalhães, A
Data de Publicação: 2022
Outros Autores: Moreira, I, Pinheiro, S, Borba, A
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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spelling 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
dc.format.none.fl_str_mv application/pdf
dc.publisher.none.fl_str_mv Ordem dos Médicos
publisher.none.fl_str_mv 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
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
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