Childhood dermatomyositis: Clinical course of 36 patients treated with low doses of corticosteroids

• Published in: European journal of paediatric neurology Vol. 2; no. 4; pp. 205 - 211
• Main Authors: Tabarki, Brahim, Ponsot, Gerard, Prieur, Anne-Marie, Tardieu, Marc
• Format: Journal Article
• Language: English
• Published: England Elsevier Ltd 1998
• Subjects: Adolescent; Adrenal Cortex Hormones - administration & dosage; Adrenal Cortex Hormones - therapeutic use; Child; Child, Preschool; Creatine kinase; Creatine Kinase - blood; Cyclosporine - administration & dosage; Cyclosporine - therapeutic use; Dermatomyositis; Dermatomyositis - blood; Dermatomyositis - drug therapy; Dose-Response Relationship, Drug; Female; Follow-Up Studies; Humans; Immunoglobulins, Intravenous - administration & dosage; Immunoglobulins, Intravenous - therapeutic use; Male; Muscle; Myosite; Retrospective Studies; Treatment Outcome; Muscle; Creatine kinase; Myosite; Dermatomyositis
• ISSN: 1090-3798; 1532-2130
• DOI: 10.1016/S1090-3798(98)80021-4

Thirty-six patients with juvenile dermatomyositis, seen consecutively between 1983 and 1996 and treated initially with low doses of corticosteroids (prednisolone 1 mg/kg/day), were studied retrospectively to evaluate their long-term evolution and to identify factors predictive of the functional outcome. After a mean follow-up of 4.9 years, 28 (78%) of the patients were well without functional impairment; five patients had inactive disease but with persisting disabilities; and three patients had active disease despite several years of treatment. Fifteen children (42%) developed dystrophic calcifications which, in five of these patients, interfered with functions. These patients treated for juvenile dermatomyositis with a low dose corticosteroid regimen had an evolution identical to that of the published series of patients treated with higher doses and probably had a better quality of life. The best predictors of good functional recovery and minimal calcinosis were early treatment after the onset of symptoms and low creatine kinase serum level at the time of diagnosis.