title: Idiopathic Pulmonary Fibrosis: Current Status, Recent Progress, and Emerging Targets.
abstract: 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.
authors.affiliation: School of Pharmacy, College of Pharmacy, Taipei Medical University , 250 Wuxing Street, Taipei 11031, Taiwan.
authors.forename: Yi-Min
authors.initials: YM
authors.lastname: Liu
authors.affiliation: School of Pharmacy, College of Pharmacy, Taipei Medical University , 250 Wuxing Street, Taipei 11031, Taiwan.
authors.forename: Kunal
authors.initials: K
authors.lastname: Nepali
authors.affiliation: School of Pharmacy, College of Pharmacy, Taipei Medical University , 250 Wuxing Street, Taipei 11031, Taiwan.
authors.forename: Jing-Ping
authors.identifier: 0000-0002-3775-6405
authors.initials: JP
authors.lastname: Liou
mesh_terms: D000818:Animals; D000893:Anti-Inflammatory Agents / Q000627:therapeutic use; D000925:Anticoagulants / Q000627:therapeutic use; D000975:Antioxidants / Q000627:therapeutic use; D017209:Apoptosis / Q000187:drug effects; D006801:Humans; D054990:Idiopathic Pulmonary Fibrosis / Q000188:drug therapy* / Q000276:immunology / Q000473:pathology / Q000503:physiopathology; D007211:Indoles / Q000627:therapeutic use; D000074624:NADPH Oxidase 1; D000074663:NADPH Oxidase 4; D019255:NADPH Oxidases / Q000037:antagonists & inhibitors; D010330:Patents as Topic; D047428:Protein Kinase Inhibitors / Q000627:therapeutic use; D011728:Pyridones / Q000627:therapeutic use; D049368:Receptors, Lysophosphatidic Acid / Q000037:antagonists & inhibitors
chemical_list: D000893:Anti-Inflammatory Agents; D000925:Anticoagulants; D000975:Antioxidants; D007211:Indoles; C000609549:LPAR1 protein, human; D047428:Protein Kinase Inhibitors; D011728:Pyridones; D049368:Receptors, Lysophosphatidic Acid; C093844:pirfenidone; D000074624:NADPH Oxidase 1; D000074663:NADPH Oxidase 4; D019255:NADPH Oxidases; C402799:NOX1 protein, human; C498910:NOX4 protein, human; C530716:nintedanib