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Type: Artigo
Title: A Brazilian registry of juvenile dermatomyositis : onset features and classification of 189 cases
Author: Sato, JO
Sallum, A.M.E
Ferriani, V.P.L.
Marini, R.
Sacchetti, S.B.
Okuda, E.M.
De Carvalho, J.F.
Pereira, R.M.R.
Len, C.A.
Terreri, M.T.R.A.
Lotufo, S.A.
Romanelli, P.R.S.
Ramos, V.C.S.
Hilario, M.O.E.
Silva, C.A.A.
Corrente, J.E.
Saad-Magalhaes, C.
Abstract: To describe onset features, classification and treatment of juvenile dermatomyositis (JDM) and juvenile polymyositis (JPM) from a multicentre registry.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.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%.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
Subject: Dermatomiosite
Country: Itália
Editor: Pacini
Rights: Fechado
Date Issue: 2009
Appears in Collections:FCM - Artigos e Outros Documentos

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