A Brazilian registry of juvenile dermatomyositis: Onset features and classification of 189 cases

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Autor(es): dc.contributorUniversidade Estadual Paulista (UNESP)-
Autor(es): dc.contributorUniversidade de São Paulo (USP)-
Autor(es): dc.contributorUniversidade Estadual de Campinas (UNICAMP)-
Autor(es): dc.contributorFaculdade de Ciências Médicas da Santa Casa de São Paulo-
Autor(es): dc.contributorUniversidade Federal de São Paulo (UNIFESP)-
Autor(es): dc.contributorHospital Municipal Infantil Menino Jesus-
Autor(es): dc.contributorPontifícia Universidade Católica (PUC) de São Paulo-
Autor(es): dc.creatorSato, Juliana De Oliveira-
Autor(es): dc.creatorSallum, Adriana Maluf Elias-
Autor(es): dc.creatorFerriani, Virginia Paes Leme-
Autor(es): dc.creatorMarini, Roberto-
Autor(es): dc.creatorSacchetti, Silvana Brasília-
Autor(es): dc.creatorOkuda, Eunice Mitico-
Autor(es): dc.creatorDe Carvalho, Jozélio Freire-
Autor(es): dc.creatorPereira, Rosa Maria Rodrigues-
Autor(es): dc.creatorLen, Claudio Arnaldo-
Autor(es): dc.creatorTerreri, Maria Teresa R.A.-
Autor(es): dc.creatorLotufo, Simone Andrade-
Autor(es): dc.creatorRomanelli, Paulo Roberto Stocco-
Autor(es): dc.creatorRamos, Valéria Cristina Santucci-
Autor(es): dc.creatorHilário, Maria Odete Esteves-
Autor(es): dc.creatorSilva, Clóvis Artur Almeida-
Autor(es): dc.creatorCorrente, José Eduardo-
Autor(es): dc.creatorSaad-Magalhães, Cláudia-
Data de aceite: dc.date.accessioned2025-08-21T16:33:25Z-
Data de disponibilização: dc.date.available2025-08-21T16:33:25Z-
Data de envio: dc.date.issued2022-04-28-
Data de envio: dc.date.issued2022-04-28-
Data de envio: dc.date.issued2009-12-01-
Fonte completa do material: dc.identifierhttp://hdl.handle.net/11449/225773-
Fonte: dc.identifier.urihttp://educapes.capes.gov.br/handle/11449/225773-
Descrição: dc.descriptionObjective: To describe onset features, classification and treatment of juvenile dermatomyositis (JDM) and juvenile polymyositis (JPM) from a multicentre registry. Methods: Inclusion criteria were onset age lower than 18 years and a diagnosis of any idiopathic inflammatory myopathy (IIM) by attending physician. Bohan & Peter (1975) criteria categorisation was established by a scoring algorithm to define JDM and JPM based on clinical protocol data. Results: Of the 189 cases included, 178 were classified as JDM, 9 as JPM (19.8: 1) and 2 did not fit the criteria; 6.9% had features of chronic arthritis and connective tissue disease overlap. Diagnosis classification agreement occurred in 66.1%. Median onset age was 7 years, median follow-up duration was 3.6 years. Malignancy was described in 2 (1.1%) cases. Muscle weakness occurred in 95.8%; heliotrope rash 83.5%; Gottron plaques 83.1%; 92% had at least one abnormal muscle enzyme result. Muscle biopsy performed in 74.6% was abnormal in 91.5% and electromyogram performed in 39.2% resulted abnormal in 93.2%. Logistic regression analysis was done in 66 cases with all parameters assessed and only aldolase resulted significant, as independent variable for definite JDM (OR=5.4, 95%CI 1.2-24.4, p=0.03). Regarding treatment, 97.9% received steroids; 72% had in addition at least one: methotrexate (75.7%), hydroxychloroquine (64.7%), cyclosporine A (20.6%), IV immunoglobulin (20.6%), azathioprine (10.3%) or cyclophosphamide (9.6%). In this series 24.3% developed calcinosis and mortality rate was 4.2%. Conclusion: Evaluation of predefined criteria set for a valid diagnosis indicated aldolase as the most important parameter associated with definite JDM category. In practice, prednisone-methotrexate combination was the most indicated treatment. © Copyright Clinical and Experimental Rheumatology 2009.-
Descrição: dc.descriptionDepartment of Paediatrics Faculdade de Medicina de Botucatu Universidade Estradual Paulista (UNESP), 18 618-970 Botucatu, São Paulo-
Descrição: dc.descriptionInstituto da Criança FMUSP-
Descrição: dc.descriptionFaculdade de Medicina de Ribeirão Preto USP-
Descrição: dc.descriptionFaculdade de Ciências Médicas Universidade Estadual de Campinas UNICAMP-
Descrição: dc.descriptionFaculdade de Ciências Médicas da Santa Casa de São Paulo-
Descrição: dc.descriptionDisciplina de Reumatologia FMUSP-
Descrição: dc.descriptionEscola Paulista de Medicina UNIFESP-
Descrição: dc.descriptionHospital Municipal Infantil Menino Jesus, São Paulo-
Descrição: dc.descriptionDepartamento de Reumatologia Pontifícia Universidade Católica (PUC) de São Paulo-
Descrição: dc.descriptionDepartment of Paediatrics Faculdade de Medicina de Botucatu Universidade Estradual Paulista (UNESP), 18 618-970 Botucatu, São Paulo-
Formato: dc.format1031-1038-
Idioma: dc.languageen-
Relação: dc.relationClinical and Experimental Rheumatology-
???dc.source???: dc.sourceScopus-
Palavras-chave: dc.subjectIdiopathic inflammatory myopathy-
Palavras-chave: dc.subjectJuvenile dermatomyositis-
Palavras-chave: dc.subjectJuvenile polymyositis-
Palavras-chave: dc.subjectMethotrexate-
Palavras-chave: dc.subjectSteroids-
Título: dc.titleA Brazilian registry of juvenile dermatomyositis: Onset features and classification of 189 cases-
Tipo de arquivo: dc.typelivro digital-
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