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

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...

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Bibliographic Details
Authors: Sato, Juliana De Oliveira [UNESP], Sallum, Adriana Maluf Elias, Ferriani, Virginia Paes Leme, Marini, Roberto, Sacchetti, Silvana Brasília, Okuda, Eunice Mitico, De Carvalho, Jozélio Freire, Pereira, Rosa Maria Rodrigues, Len, Claudio Arnaldo, Terreri, Maria Teresa R.A., Lotufo, Simone Andrade, Romanelli, Paulo Roberto Stocco, Ramos, Valéria Cristina Santucci, Hilário, Maria Odete Esteves, Silva, Clóvis Artur Almeida, Corrente, José Eduardo [UNESP], Saad-Magalhães, Cláudia [UNESP]
Format: article
Status:Published version
Publication Date:2009
Country:Brasil
Institution:Universidade Estadual Paulista (UNESP)
Repository:Repositório Institucional da UNESP
Language:English
OAI Identifier:oai:repositorio.unesp.br:11449/225773
Online Access:http://hdl.handle.net/11449/225773
Access Level:Open access
Keyword:Idiopathic inflammatory myopathy
Juvenile dermatomyositis
Juvenile polymyositis
Methotrexate
Steroids
Description
Summary: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 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%. Conclusion: 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. © Copyright Clinical and Experimental Rheumatology 2009.

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