When idiopathic inflammatory myopathies (IIM) affect the lungs, the consequences can be serious. ILD is not only common in IIM, but also one of the leading causes of death, contributing to up to 80% of mortality in this patient group. For rheumatologists, two subsets stand out: anti-synthetase syndrome (ASyS) and anti-MDA-5 positive dermatomyositis (MDA5-DM). These patients present with distinct clinical clues, very different disease trajectories, and unique treatment challenges. Recognizing ILD early and acting decisively can make the difference between stabilizing a chronic course and facing a rapidly progressive, often fatal decline.