title: Targeted Therapy for Idiopathic Pulmonary Fibrosis: Where To Now?
abstract: Idiopathic pulmonary fibrosis (IPF) is an aging-associated, recalcitrant lung disease with historically limited therapeutic options. The recent approval of two drugs, pirfenidone and nintedanib, by the US Food and Drug Administration in 2014 has heralded a new era in its management. Both drugs have demonstrated efficacy in phase III clinical trials by retarding the rate of progression of IPF; neither drug appears to be able to completely arrest disease progression. Advances in the understanding of IPF pathobiology have led to an unprecedented expansion in the number of potential therapeutic targets. Drugs targeting several of these are under investigation in various stages of clinical development. Here, we provide a brief overview of the drugs that are currently approved and others in phase II clinical trials. Future therapeutic opportunities that target novel pathways, including some that are associated with the biology of aging, are examined. A multi-targeted approach, potentially with combination therapies, and identification of individual patients (or subsets of patients) who may respond more favourably to specific agents are likely to be more effective.
authors.affiliation: Division of Pulmonary, Allergy and Critical Care Medicine, Department of Medicine, University of Alabama at Birmingham, 1900 University Blvd THT 422, Birmingham, AL, 35294-2180, USA.
authors.forename: Sunad
authors.initials: S
authors.lastname: Rangarajan
authors.affiliation: Division of Pulmonary, Allergy and Critical Care Medicine, Department of Medicine, University of Alabama at Birmingham, 1900 University Blvd THT 422, Birmingham, AL, 35294-2180, USA.
authors.forename: Morgan L
authors.initials: ML
authors.lastname: Locy
authors.affiliation: Division of Pulmonary, Allergy and Critical Care Medicine, Department of Medicine, University of Alabama at Birmingham, 1900 University Blvd THT 422, Birmingham, AL, 35294-2180, USA.
authors.forename: Tracy R
authors.initials: TR
authors.lastname: Luckhardt
authors.affiliation: Division of Pulmonary, Allergy and Critical Care Medicine, Department of Medicine, University of Alabama at Birmingham, 1900 University Blvd THT 422, Birmingham, AL, 35294-2180, USA. vjthan@uab.edu.
authors.forename: Victor J
authors.initials: VJ
authors.lastname: Thannickal
mesh_terms: D000375:Aging / Q000187:drug effects* / Q000378:metabolism / Q000473:pathology; D017322:Clinical Trials, Phase II as Topic; D017277:Drug Approval; D006801:Humans; D054990:Idiopathic Pulmonary Fibrosis / Q000188:drug therapy* / Q000378:metabolism / Q000473:pathology; D007211:Indoles / Q000008:administration & dosage / Q000009:adverse effects / Q000493:pharmacokinetics / Q000627:therapeutic use*; D058990:Molecular Targeted Therapy; D011728:Pyridones / Q000008:administration & dosage / Q000009:adverse effects / Q000493:pharmacokinetics / Q000627:therapeutic use*
chemical_list: D007211:Indoles; D011728:Pyridones; C093844:pirfenidone; C530716:nintedanib