Several factors have been identified as predictors of a poor prognosis in patients with IIM-ILD, including acute onset of ILD, lower forced vital capacity (FVC) % predicted at ILD onset, positive anti-Ro-52 antibody, positive anti-MDA5 antibody, and the presence of usual interstitial pneumonia (UIP) (8–11). Here, IFIH1 is linked to interstitial lung disease.