A combined immunosuppressive treatment regimen of high-dose glucocorticoids, tacrolimus and intravenous cyclophosphamide was effective in a Japanese cohort of 29 patients with anti-melanoma differentiation-associated gene 5 (MDA5)+ dermatomyositis-associated interstitial lung disease (ILD) in a prospective study. The 6-month survival of patients receiving combined immunosuppression was higher than that of a historical cohort of 15 patients who received step-up therapy of high-dose glucocorticoids with additional immunosuppressants (89% versus 33%, respectively).