title: New insights into the pathogenesis and treatment of idiopathic pulmonary fibrosis.
abstract: Idiopathic pulmonary fibrosis (IPF) is the most common and lethal of the idiopathic interstitial pneumonias. There are currently no effective pharmacological therapies approved for the treatment of IPF. Despite the focus on targeting fibrogenic pathways, recent clinical trials have been largely disappointing. Progress is being made in elucidating key cellular processes and molecular pathways critical to IPF pathogenesis, and this should facilitate the development of more effective therapeutics for this recalcitrant disease. Emerging pathobiological concepts include the role of aging and cellular senescence, oxidative stress, endoplasmic reticulum stress, cellular plasticity, microRNAs and mechanotransduction. Therapeutic approaches that target molecular pathways to modulate aberrant cellular phenotypes and promote tissue homeostasis in the lung must be developed. Heterogeneity in biological and clinical phenotypes of IPF warrants a personalized medicine approach to diagnosis and treatment of this lung disorder.
authors.affiliation: Division of Pulmonary, Allergy and Critical Care Medicine, Department of Medicine, University of Alabama at Birmingham, USA.
authors.forename: Qiang
authors.initials: Q
authors.lastname: Ding
authors.forename: Tracy
authors.initials: T
authors.lastname: Luckhardt
authors.forename: Louise
authors.initials: L
authors.lastname: Hecker
authors.forename: Yong
authors.initials: Y
authors.lastname: Zhou
authors.forename: Gang
authors.initials: G
authors.lastname: Liu
authors.forename: Veena B
authors.initials: VB
authors.lastname: Antony
authors.forename: Joao
authors.initials: J
authors.lastname: deAndrade
authors.forename: Victor J
authors.initials: VJ
authors.lastname: Thannickal
mesh_terms: D000818:Animals; D006801:Humans; D054990:Idiopathic Pulmonary Fibrosis / Q000453:epidemiology / Q000209:etiology* / Q000378:metabolism / Q000473:pathology / Q000628:therapy*; D058990:Molecular Targeted Therapy / Q000639:trends; D012307:Risk Factors