Idiopathic Pulmonary Fibrosis: Current Status, Recent Progress, and Emerging Targets

Idiopathic pulmonary fibrosis (IPF), a chronic and progressive fibrosing interstitial pneumonia, is a fatal lung disease with a median survival time of 3-5 years. Problems in accurate diagnosis, poor prognosis, limited clinical therapy, and high mortality rate together demonstrate that the development of efficient therapeutic strategies for IPF is an important future endeavor. Deeper understanding of pathogenesis and identification of biomarkers and pathways involved might lead in the future to the emergence of some agents as novel therapeutics for IPF. This review article presents the pathogenesis, therapeutic interventions, treatment approaches, and strategies employed for the design of antifibrotic agents for the treatment of IPF along with the patent literature from the past 10 years. With a dozen antifibrotic agents possessing exciting preclinical potential in the armory, it seems certain that some of them will advance to clinical stage investigations. The results of clinical trials for some of the new agents are also awaited to assess their benefits in terms of efficacy and survival benefits.