The causes of death in anti-MDA5 Ab+ DM patients are significantly associated with respiratory failure via RPILD,[3,4] and lung specimens at autopsy exhibit histopathological diffuse alveolar damage (DAD).[5] However, further investigation is needed to understand the pathological conditions associated with deterioration in anti-MDA5 Ab+ DM patients with RPILD. The gene discussed is IFIH1; the disease is dermatomyositis.