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

• Main Author: Sato, J. O.
• Publication Date: 2009
• Other Authors: Sallum, A. M. E., Ferriani, Virginia Paes Leme [UNIFESP], Marini, R., Sacchetti, S. B., Okuda, E. M., Carvalho, J. F., Pereira, R. M. R., Len, Claudio Arnaldo [UNIFESP], Terreri, Maria Teresa Ramos Ascensão [UNIFESP], Lotufo, S. A., Romanelli, P. R., Ramos, V. C. S., Hilário, Maria Odete Esteves [UNIFESP], Silva, C. A., Corrente, J. E., Saad-Magalhaes, C., Rheumatol Comm Sao Paulo Paediat S
• Format: Article
• Language: eng
• Source: Repositório Institucional da UNIFESP
• Download full: http://www.clinexprheumatol.org/article.asp?a=7; http://repositorio.unifesp.br/handle/11600/43323
• topic: Idiopathic inflammatory myopathy; juvenile dermatomyositis; juvenile polymyositis; methotrexate; steroids

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.