OBJECTIVE: Cyclosporin A (CyA) has been reported to be an effective treatment 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: Better clinical control of the disease was obtained in all cases and corticosteroid doses could be markedly decreased or stopped. 4 patients relapsed, 3 after discontinuation of CyA, but recovered after its reinitiation. 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.
OBJECTIVE:Cyclosporin A (CyA) has been reported to be an effective treatment 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: Better clinical control of the disease was obtained in all cases and corticosteroid doses could be markedly decreased or stopped. 4 patients relapsed, 3 after discontinuation of CyA, but recovered after its reinitiation. 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.
Authors: C H Spencer; K Rouster-Stevens; H Gewanter; G Syverson; R Modica; K Schmidt; H Emery; C Wallace; S Grevich; K Nanda; Y D Zhao; S Shenoi; S Tarvin; S Hong; C Lindsley; J E Weiss; M Passo; K Ede; A Brown; K Ardalan; W Bernal; M L Stoll; B Lang; R Carrasco; C Agaiar; L Feller; H Bukulmez; R Vehe; H Kim; H Schmeling; D Gerstbacher; M Hoeltzel; B Eberhard; R Sundel; S Kim; A M Huber; A Patwardhan Journal: Pediatr Rheumatol Online J Date: 2017-06-13 Impact factor: 3.054