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Archivos de Pediatría del Uruguay
Print version ISSN 0004-0584On-line version ISSN 1688-1249
Abstract
CAGGIANI, Marina et al. Juvenile dermatomyositis: 13 years of experience in a tertiary care hospital. Analysis of 17 clinical cases. Arch. Pediatr. Urug. [online]. 2018, vol.89, n.2, pp.99-107. ISSN 0004-0584. https://doi.org/10.31134/ap.89.2.4.
Juvenile dermatomyositis (JDM) is an acquired inflammatory myopathy with an immunologic basis and characteristic cutaneous rash. The aim of this article is to describe clinical features and most important exams of a group of 17 patients with JDM their evolution and treatment.
Methods:
children with JDM recruited from Connective tissue Diseases Office of Pereira Rossell Hospital from 1/10/2003 through 1/4/2017 were studied.
Results:
the most frequent features were: characteristic cutaneous rash, muscle weakness, systemic symptoms, gastrointestinal and respiratory manifestations. The diagnostic investigations showed an increase serum muscle enzymes in all patients. The nuclear magnetic resonance, electromyogram and muscle biopsy resulted abnormal in all the investigated cases. Treatment was based on corticosteroids and immunosuppressive drugs being methotrexate the preferred drug. In severe or refractory cases cyclophosphamide, human gammaglobulin or cyclosporine were associated. Median treatment length was 3 years 10 months Remission was achieved in 47 percent. The evolution was monophasic in 15.4 percent, polyphasic in 7.7 and chronic in 77 percent. No deaths were registered neither malignant associated diseases
Conclusions:
JDM is an infrequent illness. Most of the patients had chronic evolution. This obliges to prolonged immunosuppression with its adverse effects. Remission was achieved in 47% of the cases. No deaths were registered in this population.
Keywords : Dermatomyositis; Children; Signs and symptoms; Clinical evolution.
