title: Current and emerging drugs for idiopathic pulmonary fibrosis.
abstract: Idiopathic pulmonary fibrosis is a chronic, progressive and often fatal form of interstitial lung disease. It is characterized by injury with loss of lung epithelial cells and abnormal tissue repair, resulting in abnormal accumulation of fibroblasts and myofibroblasts, deposition of extracellular matrix and distortion of lung architecture, leading to respiratory failure. This lethal lung disorder continues to pose major clinical challenges as an effective therapeutic regimen has yet to be developed. In this report, therapeutic strategies are reviewed, including the use of antifibrotic agents, inhibition of cytokines, leukotrienes and cytokines receptors, and molecular targeting of specific signaling pathways during fibrotic processes and angiogenesis. This article examines the body of evidence supporting present therapies and reviews the newer agents being tested in patients with idiopathic pulmonary fibrosis.
authors.affiliation: University of Michigan Medical School, Division of Pulmonary and Critical Care Medicine, Department of Internal Medicine, 2215 Fuller Road, 11R, Ann Arbor, MI 48105, USA. nazy@umich.edu
authors.forename: Mehrnaz
authors.initials: M
authors.lastname: Gharaee-Kermani
authors.forename: Biao
authors.initials: B
authors.lastname: Hu
authors.forename: Victor J
authors.initials: VJ
authors.lastname: Thannickal
authors.forename: Sem H
authors.initials: SH
authors.lastname: Phan
authors.forename: Margaret R
authors.initials: MR
authors.lastname: Gyetko
mesh_terms: D020533:Angiogenesis Inhibitors / Q000008:administration & dosage / Q000494:pharmacology / Q000627:therapeutic use; D000818:Animals; D000894:Anti-Inflammatory Agents, Non-Steroidal / Q000008:administration & dosage / Q000494:pharmacology / Q000627:therapeutic use; D000975:Antioxidants / Q000008:administration & dosage / Q000494:pharmacology / Q000627:therapeutic use; D002986:Clinical Trials as Topic; D015195:Drug Design; D004345:Drug Industry; D006801:Humans; D007155:Immunologic Factors / Q000008:administration & dosage / Q000494:pharmacology / Q000627:therapeutic use; D011658:Pulmonary Fibrosis / Q000188:drug therapy* / Q000209:etiology / Q000276:immunology
chemical_list: D020533:Angiogenesis Inhibitors; D000894:Anti-Inflammatory Agents, Non-Steroidal; D000975:Antioxidants; D007155:Immunologic Factors