Navigating the Challenges of Treating Autoimmune-Inflammatory Myopathy-Related Interstitial Lung Disease With Nintedanib

Autoimmune-inflammatory myopathy-related interstitial lung disease (AIM-ILD) encompasses a heterogeneous spectrum of pulmonary involvement, with patterns ranging from predominantly inflammatory to clearly fibrosing, and is characterized by a highly variable clinical course and prognosis. Clinicians continue to face challenges in AIM-ILD because of its rarity, clinical heterogeneity, and the limited evidence base that guides current practice. When interstitial lung disease arises secondary to autoimmune-inflammatory myopathy—such as dermatomyositis or polymyositis—the disease can progress rapidly and may result in respiratory failure. Immunosuppressive agents remain the cornerstone of treatment; however, in patients who progress despite standard therapy, antifibrotic treatment with nintedanib may offer a viable therapeutic strategy. Early evidence suggests promising results in rapidly progressive forms; nevertheless, robust data remain limited.