Abstract
Mizoribine (MZR) is a second-line immunosuppressive drug that has been used in Japan to prevent rejection of renal allografts, and also for treatment of rheumatoid arthritis, nephrotic syndrome, immunoglobulin A nephropathy and systemic lupus erythematosus. We report a 47-year-old woman who was diagnosed as having interstitial pneumonia and dermatomyositis. She was initially treated with corticosteroid and azathioprine, but during tapering of the corticosteroid, the interstitial pneumonia recurred. She was therefore treated with mizoribine and corticosteroid, and this led to complete resolution of the disease. In this case, mizoribine was used as a second-line treatment and steroid-tapering agent. Mizoribine appears to be a useful agent for treatment of chronic interstitial pneumonia in polymyositis/dermatomyositis, and the present case is the first to have been reported in which the drug has been used successfully for treatment of interstitial pneumonia with dermatomyositis.