Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive interstitial lung disease characterised by irreversible fibrotic remodelling and a poor prognosis. The typical survival rate is 3-5 years after diagnosis. Although considerable progress has been made in understanding its underlying biology, early detection remains difficult and available treatments provide only limited benefit. Current evidence points to IPF as a multifactorial disease driven by the interplay of alveolar epithelial cell injury, impaired repair mechanisms, immune dysregulation, particularly macrophage polarisation, fibroblast activation, and oxidative stress. These processes converge to promote excessive extracellular matrix deposition and progressive decline in lung function. In this review, we bring together current insights into the cellular and molecular mechanisms of IPF, with particular attention to interactions among the epithelial, immune, and mesenchymal compartments. We also discuss how genetic susceptibility, epigenetic regulation, and environmental exposures contribute to disease onset and patient variability. In addition to established antifibrotic therapies such as pirfenidone and nintedanib, we examine emerging approaches including targeted molecular treatments, antioxidant strategies, and regenerative therapies. Despite promising developments, translating mechanistic findings into meaningful clinical outcomes remains a major challenge. Moving forward, improved biomarkers, better patient stratification, and multi-targeted therapeutic strategies will be essential to achieving more effective and personalized treatment for IPF.
Zhang et al. (Mon,) studied this question.