CYCLOSPORINE-A THERAPY IN REFRACTORY JUVENILE DERMATOMYOSITIS - EXPERIENCE AND LONG-TERM FOLLOW-UP OF 6 CASES

Objective. Cyclosporin A (CyA) has been reported to be an effective tr eatment of adult polymyositis (PM) and dermatomyositis (DM) refractory to corticosteroid therapy. We aimed to determine the effectiveness of CyA in the treatment of juvenile dermatomyositis (JDM) refractory to corticosteroids and cytotoxic agents, Methods, Retrospective study of 6 patients with refractory JDM based on medical charts. Results, Bette r clinical control of the disease was obtained in all cases and cortic osteroid doses could be markedly decreased or stopped. 4 patients rela psed, 3 after discontinuation of CyA, but recovered after its reinitia tion. Trough CyA levels were maintained between 51 and 247 ng/ml, and side effects were rare and minor during a mean followup of 51.5 months . Conclusion. CyA seems to be an effective treatment of JDM and should be considered in case of severe or refractory disease.