 Anti-melanoma differentiation associated protein 5, MDA5, antibody-positive dermatomyositis, MDA5DM, is often accompanied by rapidly progressive interstitial lung disease, RPALD, which has a poor prognosis despite intensive treatment with a combination of high-dose glucocorticoids and single-conventional immunosuppressants. The triple therapy, high-dose glucocorticoids, cyclophosphamide and tochrolimus, was found to be more effective than a combination of high-dose glucocorticoids and stepwise addition of immunosuppressants. Additionally, the efficacy of tophacetin of 10 mg per day, milligram day, for MDA5DM with RPALD that is resistant to the triple therapy was suggested. However, the effectiveness of these treatments was only compared to the historical control. Furthermore, there were still some patients who did not respond to the triple therapy or the stepwise addition of immunosuppressants. In this case series, we This article was authored by Keikeda, Taro Iwamoto, Chansuke Furuta and others. We are article.tv, links in the description below.