Given the limited efficacy of conventional therapies and the overall poor prognosis of anti-MDA5-positive dermatomyositis (MDA5<sup>+</sup> DM) with RP-ILD, our observations-together with prior reports-support lung transplantation, with extracorporeal membrane oxygenation (ECMO) as a bridge when necessary, as a promising and reliable therapeutic strategy. Here, IFIH1 is linked to interstitial lung disease.