Abstract
We experienced 3 cases of refractory juvenile dermatomyositis that intravenous immune globulin
(IVIG)has responded. Case l is 5-y-o boy. He was treated with intravenous methylprednisolone(IVMP),
oral corticosteroid and methotrexate at the onset,and he had maintained remission. After 10 months, he had a
relapse. We underwent intravenous cyclophosphamide(IVCYC)in addition to the change from methotrexate
to azathioprine, but he did not respond. Therefore,we performed IVIG, added methotrexate and changed from
azathioprine to cyclosporin A,and he was in remission.After that he relapsed,we underwent IVIG again and
changed from cyclosporin A to tacrolimus. Case 2 is 11-y-o girl. Her symptoms were improved IVMP and oral
corticosteroid,but worsened according as decreasing corticosteroid after 5 months from the start of treatment.
Despite increasing corticosteroid and administrating methotrexate she was not improved.Accordingly,we
performed IVIG and added cyclosporin A,and she was in remission. After 4 months,on account of relapse she
was administered IVIG again. Since then,she has maintained in remission(she has on no medication after 4
years and 5 months after last IVIG). Case 3 is 15-y-o boy. He was complicated by interstitial pneumonia. We
treated him with IVMP,oral corticosteroid and azathioprine.We changed from azathioprine to cyclosporin
A,performed IVCYC(8 times)and replaced cyclosporine A with tacrolimus after IVCYC, for interstitial
pneumonia. His myositis relapse as decreasing corticosteroid,and we performed IVIG, increased corticosteroid
and added methotrexate. Then he has been in remission.
Their symptoms of myositis were in effect of IVIG immediately and also observed in the effects of
cutaneous symptoms as well.