The median duration from DM onset to HPS development was 3 months, and the fatality rate was 50%.[11] Based on the results of this study, HPS complications in patients with anti-MDA5 antibody-positive DM are presumed to be frequent in East Asia, similar to those in patients with RP-ILD. This evidence concerns the gene IFIH1 and Hermansky-Pudlak syndrome.