Erasmus Syndrome: An Underrecognized Entity

Detalhes bibliográficos
Autor(a) principal: Magalhães, Ana
Data de Publicação: 2022
Outros Autores: Moreira, Inês, Pinheiro, Sofia, Borba, Alexandra
Tipo de documento: Artigo
Idioma: eng
Título da fonte: Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos)
Texto Completo: https://www.actamedicaportuguesa.com/revista/index.php/amp/article/view/16896
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 ReconhecidaConnective Tissue DiseasesLung Diseases, InterstitialSilicosisScleroderma, SystemicDoenças Pulmonares IntersticiaisDoenças do Tecido ConjuntivoEscleroderma SistémicoSilicoseWe 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 Médicos2022-05-13info:eu-repo/semantics/publishedVersioninfo:eu-repo/semantics/articleapplication/pdfhttps://www.actamedicaportuguesa.com/revista/index.php/amp/article/view/16896Acta Médica Portuguesa; Vol. 36 No. 2 (2023): February; 122-126Acta Médica Portuguesa; Vol. 36 N.º 2 (2023): Fevereiro; 122-1261646-07580870-399Xreponame:Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos)instname:Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informaçãoinstacron:RCAAPenghttps://www.actamedicaportuguesa.com/revista/index.php/amp/article/view/16896https://www.actamedicaportuguesa.com/revista/index.php/amp/article/view/16896/6637Direitos de Autor (c) 2022 Acta Médica Portuguesainfo:eu-repo/semantics/openAccessMagalhães, AnaMoreira, InêsPinheiro, SofiaBorba, Alexandra2023-02-12T03:00:34Zoai:ojs.www.actamedicaportuguesa.com:article/16896Portal AgregadorONGhttps://www.rcaap.pt/oai/openaireopendoar:71602024-03-19T16:20:50.349296Repositório 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, Ana
Connective Tissue Diseases
Lung Diseases, Interstitial
Silicosis
Scleroderma, Systemic
Doenças Pulmonares Intersticiais
Doenças do Tecido Conjuntivo
Escleroderma Sistémico
Silicose
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, Ana
author_facet Magalhães, Ana
Moreira, Inês
Pinheiro, Sofia
Borba, Alexandra
author_role author
author2 Moreira, Inês
Pinheiro, Sofia
Borba, Alexandra
author2_role author
author
author
dc.contributor.author.fl_str_mv Magalhães, Ana
Moreira, Inês
Pinheiro, Sofia
Borba, Alexandra
dc.subject.por.fl_str_mv Connective Tissue Diseases
Lung Diseases, Interstitial
Silicosis
Scleroderma, Systemic
Doenças Pulmonares Intersticiais
Doenças do Tecido Conjuntivo
Escleroderma Sistémico
Silicose
topic Connective Tissue Diseases
Lung Diseases, Interstitial
Silicosis
Scleroderma, Systemic
Doenças Pulmonares Intersticiais
Doenças do Tecido Conjuntivo
Escleroderma Sistémico
Silicose
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 2022-05-13
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 https://www.actamedicaportuguesa.com/revista/index.php/amp/article/view/16896
url https://www.actamedicaportuguesa.com/revista/index.php/amp/article/view/16896
dc.language.iso.fl_str_mv eng
language eng
dc.relation.none.fl_str_mv https://www.actamedicaportuguesa.com/revista/index.php/amp/article/view/16896
https://www.actamedicaportuguesa.com/revista/index.php/amp/article/view/16896/6637
dc.rights.driver.fl_str_mv Direitos de Autor (c) 2022 Acta Médica Portuguesa
info:eu-repo/semantics/openAccess
rights_invalid_str_mv Direitos de Autor (c) 2022 Acta Médica Portuguesa
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 Acta Médica Portuguesa; Vol. 36 No. 2 (2023): February; 122-126
Acta Médica Portuguesa; Vol. 36 N.º 2 (2023): Fevereiro; 122-126
1646-0758
0870-399X
reponame:Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos)
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instname_str Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informação
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