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Title: A Brazilian registry of juvenile dermatomyositis: onset features and classification of 189 cases
Authors: Sato, J. O.
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
Univ Estradual Paulista
Universidade de São Paulo (USP)
Universidade Estadual de Campinas (UNICAMP)
Fac Ciencias Med Santa Casa Sao Paulo
Universidade Federal de São Paulo (UNIFESP)
Hosp Municipal Infantil Menino Jesus
PUC Sao Paulo
Keywords: Idiopathic inflammatory myopathy
juvenile dermatomyositis
juvenile polymyositis
Issue Date: 1-Nov-2009
Publisher: Clinical & Exper Rheumatology
Citation: Clinical And Experimental Rheumatology. Pisa: Clinical & Exper Rheumatology, v. 27, n. 6, p. 1031-1038, 2009.
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.
ISSN: 0392-856X
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