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

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dc.contributor.author Sato, J. O.
dc.contributor.author Sallum, A. M. E.
dc.contributor.author Ferriani, Virginia Paes Leme [UNIFESP]
dc.contributor.author Marini, R.
dc.contributor.author Sacchetti, S. B.
dc.contributor.author Okuda, E. M.
dc.contributor.author Carvalho, J. F.
dc.contributor.author Pereira, R. M. R.
dc.contributor.author Len, Claudio Arnaldo [UNIFESP]
dc.contributor.author Terreri, Maria Teresa Ramos Ascensão [UNIFESP]
dc.contributor.author Lotufo, S. A.
dc.contributor.author Romanelli, P. R.
dc.contributor.author Ramos, V. C. S.
dc.contributor.author Hilário, Maria Odete Esteves [UNIFESP]
dc.contributor.author Silva, C. A.
dc.contributor.author Corrente, J. E.
dc.contributor.author Saad-Magalhaes, C.
dc.contributor.author Rheumatol Comm Sao Paulo Paediat S
dc.date.accessioned 2018-06-15T16:52:42Z
dc.date.available 2018-06-15T16:52:42Z
dc.date.issued 2009-11-01
dc.identifier http://www.clinexprheumatol.org/article.asp?a=7
dc.identifier.citation Clinical And Experimental Rheumatology. Pisa: Clinical & Exper Rheumatology, v. 27, n. 6, p. 1031-1038, 2009.
dc.identifier.issn 0392-856X
dc.identifier.uri http://repositorio.unifesp.br/11600/43323
dc.description.abstract ObjectiveTo describe onset features, classification and treatment of juvenile dermatomyositis (JDM) and juvenile polymyositis (JPM) from a multicentre registry.MethodsInclusion 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 oil clinical protocol data.ResultsOf 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%. Medial? 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%.ConclusionEvaluation of predefined criteria set for a valid diagnosis indicated aldolase as the most important parameter associated with de, methotrexate combination, was the most indicated treatment. en
dc.format.extent 1031-1038
dc.language.iso eng
dc.publisher Clinical & Exper Rheumatology
dc.relation.ispartof Clinical And Experimental Rheumatology
dc.rights Acesso aberto
dc.subject Idiopathic inflammatory myopathy en
dc.subject juvenile dermatomyositis en
dc.subject juvenile polymyositis en
dc.subject methotrexate en
dc.subject steroids en
dc.title A Brazilian registry of juvenile dermatomyositis: onset features and classification of 189 cases en
dc.type Artigo
dc.contributor.institution Univ Estradual Paulista
dc.contributor.institution Universidade de São Paulo (USP)
dc.contributor.institution Universidade Estadual de Campinas (UNICAMP)
dc.contributor.institution Fac Ciencias Med Santa Casa Sao Paulo
dc.contributor.institution Universidade Federal de São Paulo (UNIFESP)
dc.contributor.institution Hosp Municipal Infantil Menino Jesus
dc.contributor.institution PUC Sao Paulo
dc.description.affiliation Univ Estradual Paulista, UNESP, Dept Paediat, Fac Med Botucatu, BR-18618970 Sao Paulo, Brazil
dc.description.affiliation FMUSP, Inst Crianca, Sao Paulo, Brazil
dc.description.affiliation Univ Sao Paulo, Fac Med Ribeirao Preto, Sao Paulo, Brazil
dc.description.affiliation Univ Estadual Campinas, UNICAMP, Fac Ciencias Med, BR-13081970 Campinas, SP, Brazil
dc.description.affiliation Fac Ciencias Med Santa Casa Sao Paulo, Sao Paulo, Brazil
dc.description.affiliation FMUSP, Disciplina Reumatol, Sao Paulo, Brazil
dc.description.affiliation Univ Fed Sao Paulo, Escola Paulista Med, Sao Paulo, Brazil
dc.description.affiliation Hosp Municipal Infantil Menino Jesus, Sao Paulo, Brazil
dc.description.affiliation PUC Sao Paulo, Dept Reumatol, Sao Paulo, Brazil
dc.description.affiliationUnifesp Univ Fed Sao Paulo, Escola Paulista Med, Sao Paulo, Brazil
dc.description.source Web of Science
dc.identifier.wos WOS:000274264700026



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