Titre : | A Brazilian registry of juvenile dermatomyositis : onset features and classification of 189 cases |
Revue : | Clinical and experimental rheumatology, 27, 6 |
Auteurs : | Sato JO ; Sallum AME ; Ferriani VPL ; Marini R ; Sacchetti SB ; Okuda EM ; Carvalho JF ; Pereira RMR ; Len CA ; Terreri MT ; Lotufo SA ; Romanelli PR ; Ramos VCS ; Hilario MO ; Silva CA ; Corrente JE ; Saad-Magalhaes C |
Type de document : | Article |
Année de publication : | 2009 |
Pages : | p. 1031 |
Langues: | Anglais |
Mots-clés : | arthrite ; azathioprine ; biopsie musculaire ; Brésil ; calcinose ; ciclosporine ; cyclophosphamide ; dermatomyosite juvénile ; diagnostic ; électromyographie ; évolution de la maladie ; faiblesse musculaire ; immunoglobuline ; maladie du tissu conjonctif ; méthotrexate ; muscle squelettique ; pharmacothérapie ; polymyosite ; registre de malades ; tumeur maligne |
Résumé : |
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. |
Lien associé : | Texte complet en accès libre sur le site de Clinical and Experimental Rheumatology |
Pubmed / DOI : | Pubmed : 20149327 |