Anti-melanoma differentiation-related gene 5 (MDA5) antibody-positive dermatomyositis (anti-MDA5+ DM) is recognized as a distinct subtype of dermatomyositis, characterized by its frequent association with interstitial lung disease (ILD), particularly rapidly progressive ILD (RP-ILD), which is associated with a poor prognosis and high mortality. The gene discussed is IFIH1; the disease is retinitis pigmentosa 1.