Juvenile dermatomyositis : Clinical profile and disease course in 25 patients

• Published in: Clinical and experimental rheumatology Vol. 17; no. 1; pp. 115 - 118
• Main Authors: SHEHATA, R, AL-MAYOUF, S, AL-DALAAN, A, AL-MAZAID, A, AL-BALAA, S, BAHABRI, S
• Format: Conference Proceeding Journal Article
• Language: English
• Published: Pisa Clinical and Experimental Rheumatology 1999
• Subjects: Adolescent; Biological and medical sciences; Calcinosis - complications; Calcinosis - pathology; Child; Child, Preschool; Dermatologic Agents - therapeutic use; Dermatomyositis - complications; Dermatomyositis - drug therapy; Dermatomyositis - pathology; Drug Therapy, Combination; Female; Glucocorticoids - therapeutic use; Humans; Hydroxychloroquine - therapeutic use; Immunosuppressive Agents - therapeutic use; Infant; Male; Medical sciences; Methotrexate - therapeutic use; Prednisolone - therapeutic use; Retrospective Studies; Sarcoidosis. Granulomatous diseases of unproved etiology. Connective tissue diseases. Elastic tissue diseases. Vasculitis; Vasculitis - complications; Vasculitis - pathology; Human; Immunopathology; Connective tissue disease; Skin disease; Dermatomyositis; Evolutivity; Autoimmune disease; Symptomatology; Striated muscle disease; Treatment; Age of onset; Calcinosis; Systemic disease; Complication; Child
• ISSN: 0392-856X; 1593-098X

A retrospective analysis of 25 Arab patients with juvenile dermatomyositis (JDMS) was conducted between 1988 and 1996. The mean age at disease onset was 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 family history of JDMS. The clinical features included fever in 14 patients (56%), weight loss in 20 (80%), muscle weakness in all 25 (100%), and muscle 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 pigmentary changes in 12 (48%). Seventeen of the 25 patients had arthralgia (68%) and 16 patients had arthritis (64%). Gastrointestinal symptoms were noted in 19 patients (76%). Myocarditis with cardiac failure was the initial presentation of 1 patient, while 2 had conduction defect. Twelve patients (48%) had respiratory symptoms. The course of the disease was complicated by calcinosis in 10 patients (40%). All of the patients were treated with prednisone; 15 were also treated with methotrexate. The duration of follow up ranged from 6-108 months (mean 54.5 months). Twenty-three patients improved, including those who had calcinosis at the time of presentation, with a current 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 bedridden.