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|Type:||Artigo de periódico|
|Title:||A Brazilian registry of juvenile dermatomyositis: onset features and classification of 189 cases|
|Author:||SATO, J. O.|
SALLUM, A. M. E.
FERRIANI, V. P. L.
SACCHETTI, S. B.
OKUDA, E. M.
CARVALHO, J. F.
PEREIRA, R. M. R.
LEN, C. A.
TERRERI, M. T.
LOTUFO, S. A.
ROMANELLI, P. R.
RAMOS, V. C. S.
HILARIO, M. O.
SILVA, C. A.
CORRENTE, J. E.
Rheumatol Comm Sao Paulo Paediat S
|Abstract:||Objective To describe onset features, classification and treatment of juvenile dermatomyositis (JDM) and juvenile polymyositis (JPM) from a multicentre registry. Methods 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 oil clinical protocol data. Results 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%. 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%. Conclusion Evaluation 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.|
|Subject:||Idiopathic inflammatory myopathy|
|Editor:||CLINICAL & EXPER RHEUMATOLOGY|
|Appears in Collections:||FCM - Artigos e Outros Documentos|
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