Purpose of Review <p>Idiopathic inflammatory myositis (IIM) has been identified as a potential cause of interstitial lung disease (ILD). Lung involvement is one of the leading causes of mortality. Early and effective therapy improves survival rates and overall disease outcomes.</p> Recent Findings <p>Multiple pharmacological therapies have been used to manage IIM-related ILD. Corticosteroids remain the main drug utilized, with a reasonable effectiveness; however, given their adverse events profile, long-term use is limited, and steroid-sparing agents should be used. Azathioprine, mycophenolate mofetil, tacrolimus, and cyclosporine are effective in mild to moderate disease. Cyclophosphamide and rituximab have been reserved for severe and life-threatening disease. Recently, new agents such as Janus kinase (JAK) inhibitors have been used more frequently. Antifibrotic medications are used in cases of progressive pulmonary fibrosis (PFF).</p> Summary <p>To achieve a favorable therapeutic response, it is essential to select an individualized regimen based on disease characteristics, patient demographics, and clinical conditions.</p>