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

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dc.contributor.authorSato, Juliana de Oliveira [UNESP]
dc.contributor.authorSallum, A. M. E.
dc.contributor.authorFerriani, V. P. L.
dc.contributor.authorMarini, R.
dc.contributor.authorSacchetti, S. B.
dc.contributor.authorOkuda, E. M.
dc.contributor.authorCarvalho, J. F.
dc.contributor.authorPereira, R. M. R.
dc.contributor.authorLen, C. A.
dc.contributor.authorTerreri, M. T.
dc.contributor.authorLotufo, S. A.
dc.contributor.authorRomanelli, P. R.
dc.contributor.authorRamos, V. C. S.
dc.contributor.authorHilario, M. O.
dc.contributor.authorSilva, C. A.
dc.contributor.authorCorrente, José Eduardo [UNESP]
dc.contributor.authorSaad-Magalhaes, C. [UNESP]
dc.contributor.institutionUniversidade Estadual Paulista (Unesp)
dc.contributor.institutionUniversidade de São Paulo (USP)
dc.contributor.institutionUniversidade Estadual de Campinas (UNICAMP)
dc.contributor.institutionUniversidade Federal de São Paulo (UNIFESP)
dc.contributor.institutionHosp Municipal Infantil Menino Jesus
dc.contributor.institutionPontifícia Universidade Católica de São Paulo (PUC-SP)
dc.date.accessioned2014-05-20T13:38:02Z
dc.date.available2014-05-20T13:38:02Z
dc.date.issued2009-11-01
dc.description.abstractObjectiveTo 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.description.affiliationUniv Estradual Paulista, UNESP, Dept Paediat, Fac Med Botucatu, BR-18618970 São Paulo, Brazil
dc.description.affiliationFMUSP, Inst Crianca, São Paulo, Brazil
dc.description.affiliationUniv São Paulo, Fac Med Ribeirao Preto, São Paulo, Brazil
dc.description.affiliationUniv Estadual Campinas, UNICAMP, Fac Ciencias Med, BR-13081970 Campinas, SP, Brazil
dc.description.affiliationFac Ciencias Med Santa Casa São Paulo, São Paulo, Brazil
dc.description.affiliationFMUSP, Disciplina Reumatol, São Paulo, Brazil
dc.description.affiliationUniv Fed São Paulo, Escola Paulista Med, São Paulo, Brazil
dc.description.affiliationHosp Municipal Infantil Menino Jesus, São Paulo, Brazil
dc.description.affiliationPUC São Paulo, Dept Reumatol, São Paulo, Brazil
dc.description.affiliationUnespUniv Estradual Paulista, UNESP, Dept Paediat, Fac Med Botucatu, BR-18618970 São Paulo, Brazil
dc.format.extent1031-1038
dc.identifierhttp://www.ncbi.nlm.nih.gov/pubmed/20149327
dc.identifier.citationClinical and Experimental Rheumatology. Pisa: Clinical & Exper Rheumatology, v. 27, n. 6, p. 1031-1038, 2009.
dc.identifier.issn0392-856X
dc.identifier.lattes7098310008371632
dc.identifier.orcid0000-0001-5478-4996
dc.identifier.orcid0000-0002-7631-7093
dc.identifier.urihttp://hdl.handle.net/11449/13189
dc.identifier.wosWOS:000274264700026
dc.language.isoeng
dc.publisherClinical & Exper Rheumatology
dc.relation.ispartofClinical and Experimental Rheumatology
dc.relation.ispartofjcr3.201
dc.rights.accessRightsAcesso restrito
dc.sourceWeb of Science
dc.subjectIdiopathic inflammatory myopathyen
dc.subjectjuvenile dermatomyositisen
dc.subjectjuvenile polymyositisen
dc.subjectmethotrexateen
dc.subjectsteroidsen
dc.titleA Brazilian registry of juvenile dermatomyositis: onset features and classification of 189 casesen
dc.typeArtigo
dcterms.rightsHolderClinical & Exper Rheumatology
unesp.author.lattes7098310008371632[17]
unesp.author.orcid0000-0002-3723-5028[8]
unesp.author.orcid0000-0001-5478-4996[16]
unesp.author.orcid0000-0002-7631-7093[17]
unesp.campusUniversidade Estadual Paulista (Unesp), Faculdade de Medicina, Botucatupt

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