Successful Treatment with Regimen of Intravenous Gammaglobulin and Cyclophosphamide for Dermatomyositis Accompanied by Interstitial Pneumonia, Opportunistic Infection and Steroid Psychosis

Abstract

BACKGROUND: A 47-year-old Japanese woman was suffering from dermatomyositis with progressive interstitial pneumonia, which was resistant to treatment with prednisolone (Pred) and cyclosporine A (CsA). Unfortunately, the opportunistic infection and steroid psychosis made therapeutic intervention using additional immunosuppressive drugs problematic. To overcome these difficulties, we created a regimen of intravenous gammaglobulin (IVIG) and cyclophosphamide (CPM) for the treatment of this patient. METHODS: For the simultaneous treatment, IVIG-CPM was added to Pred/CsA by means of infusion of IVIG on five consecutive days per month, followed by CPM infusion on the ninth day after the last IVIG administration. The treatment was repeated for four months. RESULTS: This regimen induced almost full remission without exacerbation of the opportunistic infection or mental disturbance. CONCLUSIONS: The outcome reported here suggests that the combination therapy of Pred/CsA and IVIG-CPM appears to be useful for the treatment of dermatomyositis with pulmonary, infectious and mental complications.