Progressive Fibrosing ILD

Abstract

Progressive fibrosing interstitial lung disease (PF-ILD) is a subgroup of ILDs that present a progressive-fibrosing phenotype. Progressive fibrosis is associated with worsening respiratory symptoms, decline in lung function, limited response to immunomodulator therapies and worsening quality of life leading to early mortality. A multidisciplinary approach is essential for optimal diagnosis and management. Pirfenidone and nintedanib are the only approved drugs that show benefits in PF-ILD.