title: Novel mechanisms and treatment of idiopathic pulmonary fibrosis.
abstract: Idiopathic pulmonary fibrosis (IPF) is a devastating progressive disease of unknown etiology that carries a grim prognosis. Over the last few decades there have been significant advances in our understanding of the mechanisms that drive the fibrotic process. In this review, we discuss the natural history of IPF, recent discoveries of the genetic factors, and environmental and infectious exposures that influence the development and progression of the disease, and highlight some of the novel discoveries in our understanding of the mechanisms that govern lung fibrosis. Finally, we discuss the new and exciting therapies that are now available to manage this illness.
authors.affiliation: Department of Medicine, University of Minnesota, Minneapolis, MN 55455, USA.
authors.forename: Firas
authors.initials: F
authors.lastname: Elmufdi
authors.affiliation: Department of Medicine, University of Minnesota, Minneapolis, MN 55455, USA.
authors.forename: Craig A
authors.initials: CA
authors.lastname: Henke
authors.affiliation: Department of Medicine, University of Minnesota, Minneapolis, MN 55455, USA.
authors.forename: David M
authors.initials: DM
authors.lastname: Perlman
authors.affiliation: Department of Medicine, University of Minnesota, Minneapolis, MN 55455, USA.
authors.forename: Rade
authors.initials: R
authors.lastname: Tomic
authors.affiliation: Department of Medicine, University of Minnesota, Minneapolis, MN 55455, USA.
authors.forename: Hyun Joo
authors.initials: HJ
authors.lastname: Kim
mesh_terms: D002986:Clinical Trials as Topic; D018450:Disease Progression; D004781:Environmental Exposure; D005347:Fibroblasts / Q000276:immunology; D005764:Gastroesophageal Reflux / Q000473:pathology; D020022:Genetic Predisposition to Disease; D006801:Humans; D054990:Idiopathic Pulmonary Fibrosis / Q000235:genetics / Q000378:metabolism* / Q000628:therapy*; D007211:Indoles / Q000627:therapeutic use; D008168:Lung / Q000473:pathology; D009154:Mutation; D058628:Myofibroblasts / Q000276:immunology; D011110:Polymorphism, Genetic; D011379:Prognosis; D011728:Pyridones / Q000627:therapeutic use; D016896:Treatment Outcome
chemical_list: D007211:Indoles; D011728:Pyridones; C093844:pirfenidone; C530716:nintedanib