Paediatric Rheumatology

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

J.O. Sato, A. M.e. Sallum, V. P.l. Ferriani, R. Marini, S. B. Sacchetti, E. M.okuda, J.F. Carvalho, R. M.r. Pereira, C.A. Len, M.-T. Terreri, S.A. Lotufo, P.R. Romanelli, V. C. S. Ramos, M.O.E. Hilário, C. Silva, J. E. Corrente, C. Saad-Magalhaes

CER7
2009 Vol.27, N°6
PI 1031, PF 1038
Paediatric Rheumatology

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PMID: 20149327 [PubMed]

Received: 03/11/2008
Accepted : 05/03/2009
In Press: 06/04/2010
Published: 02/03/2010

Abstract

OBJECTIVES:
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 on 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%. 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%.CONLUSIONS: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.