Cyclosporin a therapy in refractory juvenile dermatomyositis. Experience and longterm followup of 6 cases.
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.


CONCLUSIONS
CyA seems to be an effective treatment of JDM and should be considered in case of severe or refractory disease.
Zeller
V
V
Department of Internal Medicine, Hôpital Avicenne, Université Paris-Nord, Bobigny, France.
Cohen
P
P
Prieur
A M
AM
Guillevin
L
L
D015415:Biomarkers / Q000032:analysis; D002648:Child; D016572:Cyclosporine / Q000627:therapeutic use*; D003882:Dermatomyositis / Q000188:drug therapy*; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D012189:Retrospective Studies
D015415:Biomarkers; D016572:Cyclosporine