Juvenile Dermatomyositis Forty Years On: Case Report

Detalhes bibliográficos
Autor(a) principal: Rego de Figueiredo, I
Data de Publicação: 2018
Outros Autores: Guerreiro Castro, S, Bernardino, V, Silva Nunes, J, Alves, P, Moraes-Fontes, MF
Tipo de documento: Artigo
Idioma: eng
Título da fonte: Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos)
Texto Completo: http://hdl.handle.net/10400.17/3086
Resumo: We present a case report of a 42 year old female, diagnosed at the age of 3 with Juvenile Dermatomyositis. The clinical course was severe and refractory to immunosuppressive therapy. Currently, she is mostly affected by severe muscle atrophy, large joint contractures, calcinosis, and a lipodystrophy associated metabolic syndrome with hypertriglyceridemia, insulin resistance, high total testosterone and hepatic steatosis. She developed Hodgkin´s lymphoma in the course of her disease. Personalized therapeutic choices are discussed as regards juvenile dermatomyositis complications.
id RCAP_2f89bc98a76333b2cf799dca75b91567
oai_identifier_str oai:repositorio.chlc.min-saude.pt:10400.17/3086
network_acronym_str RCAP
network_name_str Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos)
repository_id_str 7160
spelling Juvenile Dermatomyositis Forty Years On: Case ReportCalcinosis/diagnosisCalcinosis/pathologyDermatomyositis/diagnosisDermatomyositis/pathologyHCC DAUTOIMHDE IMAHCC ENDDisease ProgressionInsulin ResistanceLipodystrophy/diagnosisLipodystrophy/pathologyMetabolic Syndrome/diagnosisMetabolic Syndrome/pathologyMuscular Atrophy/diagnosisMuscular Atrophy/pathologySeverity of Illness IndexWe present a case report of a 42 year old female, diagnosed at the age of 3 with Juvenile Dermatomyositis. The clinical course was severe and refractory to immunosuppressive therapy. Currently, she is mostly affected by severe muscle atrophy, large joint contractures, calcinosis, and a lipodystrophy associated metabolic syndrome with hypertriglyceridemia, insulin resistance, high total testosterone and hepatic steatosis. She developed Hodgkin´s lymphoma in the course of her disease. Personalized therapeutic choices are discussed as regards juvenile dermatomyositis complications.ElsevierRepositório do Centro Hospitalar Universitário de Lisboa Central, EPERego de Figueiredo, IGuerreiro Castro, SBernardino, VSilva Nunes, JAlves, PMoraes-Fontes, MF2018-10-22T12:16:08Z20182018-01-01T00:00:00Zinfo:eu-repo/semantics/publishedVersioninfo:eu-repo/semantics/articleapplication/pdfhttp://hdl.handle.net/10400.17/3086engNeuromuscul Disord. 2018 Sep;28(9):791-79710.1016/j.nmd.2018.06.011info: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:RCAAP2023-03-10T09:41:11Zoai:repositorio.chlc.min-saude.pt:10400.17/3086Portal AgregadorONGhttps://www.rcaap.pt/oai/openaireopendoar:71602024-03-19T17:20:25.531444Repositório 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 Juvenile Dermatomyositis Forty Years On: Case Report
title Juvenile Dermatomyositis Forty Years On: Case Report
spellingShingle Juvenile Dermatomyositis Forty Years On: Case Report
Rego de Figueiredo, I
Calcinosis/diagnosis
Calcinosis/pathology
Dermatomyositis/diagnosis
Dermatomyositis/pathology
HCC DAUTOIM
HDE IMA
HCC END
Disease Progression
Insulin Resistance
Lipodystrophy/diagnosis
Lipodystrophy/pathology
Metabolic Syndrome/diagnosis
Metabolic Syndrome/pathology
Muscular Atrophy/diagnosis
Muscular Atrophy/pathology
Severity of Illness Index
title_short Juvenile Dermatomyositis Forty Years On: Case Report
title_full Juvenile Dermatomyositis Forty Years On: Case Report
title_fullStr Juvenile Dermatomyositis Forty Years On: Case Report
title_full_unstemmed Juvenile Dermatomyositis Forty Years On: Case Report
title_sort Juvenile Dermatomyositis Forty Years On: Case Report
author Rego de Figueiredo, I
author_facet Rego de Figueiredo, I
Guerreiro Castro, S
Bernardino, V
Silva Nunes, J
Alves, P
Moraes-Fontes, MF
author_role author
author2 Guerreiro Castro, S
Bernardino, V
Silva Nunes, J
Alves, P
Moraes-Fontes, MF
author2_role author
author
author
author
author
dc.contributor.none.fl_str_mv Repositório do Centro Hospitalar Universitário de Lisboa Central, EPE
dc.contributor.author.fl_str_mv Rego de Figueiredo, I
Guerreiro Castro, S
Bernardino, V
Silva Nunes, J
Alves, P
Moraes-Fontes, MF
dc.subject.por.fl_str_mv Calcinosis/diagnosis
Calcinosis/pathology
Dermatomyositis/diagnosis
Dermatomyositis/pathology
HCC DAUTOIM
HDE IMA
HCC END
Disease Progression
Insulin Resistance
Lipodystrophy/diagnosis
Lipodystrophy/pathology
Metabolic Syndrome/diagnosis
Metabolic Syndrome/pathology
Muscular Atrophy/diagnosis
Muscular Atrophy/pathology
Severity of Illness Index
topic Calcinosis/diagnosis
Calcinosis/pathology
Dermatomyositis/diagnosis
Dermatomyositis/pathology
HCC DAUTOIM
HDE IMA
HCC END
Disease Progression
Insulin Resistance
Lipodystrophy/diagnosis
Lipodystrophy/pathology
Metabolic Syndrome/diagnosis
Metabolic Syndrome/pathology
Muscular Atrophy/diagnosis
Muscular Atrophy/pathology
Severity of Illness Index
description We present a case report of a 42 year old female, diagnosed at the age of 3 with Juvenile Dermatomyositis. The clinical course was severe and refractory to immunosuppressive therapy. Currently, she is mostly affected by severe muscle atrophy, large joint contractures, calcinosis, and a lipodystrophy associated metabolic syndrome with hypertriglyceridemia, insulin resistance, high total testosterone and hepatic steatosis. She developed Hodgkin´s lymphoma in the course of her disease. Personalized therapeutic choices are discussed as regards juvenile dermatomyositis complications.
publishDate 2018
dc.date.none.fl_str_mv 2018-10-22T12:16:08Z
2018
2018-01-01T00:00:00Z
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/10400.17/3086
url http://hdl.handle.net/10400.17/3086
dc.language.iso.fl_str_mv eng
language eng
dc.relation.none.fl_str_mv Neuromuscul Disord. 2018 Sep;28(9):791-797
10.1016/j.nmd.2018.06.011
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 Elsevier
publisher.none.fl_str_mv Elsevier
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_ 1799131300616994816