Juvenile dermatomyositis: Clinical profile and disease course in 25 patients

A retrospective analysis of 25 Arab patients with juvenile dermatomyositis (JDMS) was conducted between 1988 and 1996. The mean age at disease onset w as 8.25 years (range 1.5 - 15 yrs) with a male: female ratio of 1.5: 1. The disease duration before diagnosis was 1 - 108 months. Two patients had a f amily history of JDMS. The clinical features included fever in 14 patients (56%), weight loss in 20 (80%), muscle weakness in all 25 (100%), and muscl e pain in 14 (56%). Skin lesions included Gottron's papules in 15 patients (60%), heliotrope in 13 (52%), erythematous malar rash in 8 (32%), and pigm entary changes in 12 (48%). Seventeen of the 25 patients had arthralgia (68 %) and 16 patients had arthritis (64%). Gastrointestinal symptoms were note d in 19 patients (76%). Myocarditis with cardiac failure was the initial pr esentation of 1 patient, while 2 had conduction defect. Twelve patients (48 %) had respiratory symptoms. The course of the disease was complicated by c alcinosis in 10 patients (40%). All of the patients were treated with predn isone; 15 were also treated with methotrexate. The duration of follow up ra nged from 6 - 108 months (mean 54.5 months). Twenty-three patients improved , including those who had calcinosis at the time of presentation, with a cu rrent muscle power of 4/5 in 10 patients (40%) and 5/5 in 13 patients (52%) . No deaths were reported in our series and no patients are currently bedri dden.