Interstitial lung diseases (ILDs) remain among the most challenging conditions in respiratory medicine, marked by clinical heterogeneity, progressive decline, and outcomes that rival advanced malignancy. Over the past decade, advances in diagnostics, therapeutics, and multidisciplinary care have reshaped the landscape, yet substantial unmet needs persist. The 2025 Special Issue on Updates in Interstitial Lung Disease brings together a collection of pragmatic reviews and original research that aim to inform the reader about emerging science and assist with clinical practice. This Special Issue highlights updated perspectives on evolving disease diagnosis and management. It addresses the practical challenges of managing ILD in the real world, with reviews focused on multidisciplinary approaches, comorbidity care, and symptom management. Alongside these reviews, original investigations present novel data on disease mechanisms, clinical phenotypes, and therapeutic strategies. Our goal is to provide clinicians and researchers with an accessible resource that informs daily practice while pointing to the future directions of the field.