<h4>Objective</h4>Spontaneous pneumomediastinum (SPM) is a life-threatening complication of anti-melanoma differentiation-associated gene 5 antibody-positive dermatomyositis (MDA5 + DM) associated interstitial lung disease (ILD), particularly in patients with rapidly progressive ILD (RP-ILD), which carries an extremely high mortality rate. Here, IFIH1 is linked to retinitis pigmentosa 1.