Abstract

Interstitial lung diseases are a heterogeneous group of disorders characterized by inflammation and/or fibrosis of the lung parenchyma, leading to a progressive loss of lung function. Idiopathic pulmonary fibrosis (IPF) is a representative model with a pathophysiology common to other types of pulmonary fibrosis. AIM: This review presents the pathophysiological mechanisms and existing and developing antifibrotic therapies of IPF. METHOD: Qualitative study through a narrative review of the pathophysiological phenomena of IPF and advances in antifibrotic therapy. RESULTS: The role of the alveolar epithelium, fibroblast/myofibroblast activity, cellular senescence and aging, immune system activity, oxidation-reduction mechanisms and genetic characteristics have been identified, which reveal the complex pathophysiology of this disease. Currently, there are only two therapies available to mitigate the effects of pulmonary fibrosis, Pirfenidone and Nintedanib, and the development and research of other antifibrotic drugs is still pending. CONCLUSIONS: There are multiple pathophysiological phenomena in IPF. Understanding them is the basis for the development and evolution of antifibrotic therapies.