title: Future directions in idiopathic pulmonary fibrosis research. An NHLBI workshop report.
abstract: The median survival of patients with idiopathic pulmonary fibrosis (IPF) continues to be approximately 3 years from the time of diagnosis, underscoring the lack of effective medical therapies for this disease. In the United States alone, approximately 40,000 patients die of this disease annually. In November 2012, the NHLBI held a workshop aimed at coordinating research efforts and accelerating the development of IPF therapies. Basic, translational, and clinical researchers gathered with representatives from the NHLBI, patient advocacy groups, pharmaceutical companies, and the U.S. Food and Drug Administration to review the current state of IPF research and identify priority areas, opportunities for collaborations, and directions for future research. The workshop was organized into groups that were tasked with assessing and making recommendations to promote progress in one of the following six critical areas of research: (1) biology of alveolar epithelial injury and aberrant repair; (2) role of extracellular matrix; (3) preclinical modeling; (4) role of inflammation and immunity; (5) genetic, epigenetic, and environmental determinants; (6) translation of discoveries into diagnostics and therapeutics. The workshop recommendations provide a basis for directing future research and strategic planning by scientific, professional, and patient communities and the NHLBI.
authors.affiliation: 1 Vanderbilt University Medical Center, Nashville, Tennessee.
authors.forename: Timothy S
authors.initials: TS
authors.lastname: Blackwell
authors.forename: Andrew M
authors.initials: AM
authors.lastname: Tager
authors.forename: Zea
authors.initials: Z
authors.lastname: Borok
authors.forename: Bethany B
authors.initials: BB
authors.lastname: Moore
authors.forename: David A
authors.initials: DA
authors.lastname: Schwartz
authors.forename: Kevin J
authors.initials: KJ
authors.lastname: Anstrom
authors.forename: Ziv
authors.initials: Z
authors.lastname: Bar-Joseph
authors.forename: Peter
authors.initials: P
authors.lastname: Bitterman
authors.forename: Michael R
authors.initials: MR
authors.lastname: Blackburn
authors.forename: William
authors.initials: W
authors.lastname: Bradford
authors.forename: Kevin K
authors.initials: KK
authors.lastname: Brown
authors.forename: Harold A
authors.initials: HA
authors.lastname: Chapman
authors.forename: Harold R
authors.initials: HR
authors.lastname: Collard
authors.forename: Gregory P
authors.initials: GP
authors.lastname: Cosgrove
authors.forename: Robin
authors.initials: R
authors.lastname: Deterding
authors.forename: Ramona
authors.initials: R
authors.lastname: Doyle
authors.forename: Kevin R
authors.initials: KR
authors.lastname: Flaherty
authors.forename: Christine Kim
authors.initials: CK
authors.lastname: Garcia
authors.forename: James S
authors.initials: JS
authors.lastname: Hagood
authors.forename: Craig A
authors.initials: CA
authors.lastname: Henke
authors.forename: Erica
authors.initials: E
authors.lastname: Herzog
authors.forename: Cory M
authors.initials: CM
authors.lastname: Hogaboam
authors.forename: Jeffrey C
authors.initials: JC
authors.lastname: Horowitz
authors.forename: Talmadge E
authors.initials: TE
authors.lastname: King
authors.forename: James E
authors.initials: JE
authors.lastname: Loyd
authors.forename: William E
authors.initials: WE
authors.lastname: Lawson
authors.forename: Clay B
authors.initials: CB
authors.lastname: Marsh
authors.forename: Paul W
authors.initials: PW
authors.lastname: Noble
authors.forename: Imre
authors.initials: I
authors.lastname: Noth
authors.forename: Dean
authors.initials: D
authors.lastname: Sheppard
authors.forename: Julie
authors.initials: J
authors.lastname: Olsson
authors.forename: Luis A
authors.initials: LA
authors.lastname: Ortiz
authors.forename: Thomas G
authors.initials: TG
authors.lastname: O'Riordan
authors.forename: Tim D
authors.initials: TD
authors.lastname: Oury
authors.forename: Ganesh
authors.initials: G
authors.lastname: Raghu
authors.forename: Jesse
authors.initials: J
authors.lastname: Roman
authors.forename: Patricia J
authors.initials: PJ
authors.lastname: Sime
authors.forename: Thomas H
authors.initials: TH
authors.lastname: Sisson
authors.forename: Daniel
authors.initials: D
authors.lastname: Tschumperlin
authors.forename: Shelia M
authors.initials: SM
authors.lastname: Violette
authors.forename: Timothy E
authors.initials: TE
authors.lastname: Weaver
authors.forename: Rebecca G
authors.initials: RG
authors.lastname: Wells
authors.forename: Eric S
authors.initials: ES
authors.lastname: White
authors.forename: Naftali
authors.initials: N
authors.lastname: Kaminski
authors.forename: Fernando J
authors.initials: FJ
authors.lastname: Martinez
authors.forename: Thomas A
authors.initials: TA
authors.lastname: Wynn
authors.forename: Victor J
authors.initials: VJ
authors.lastname: Thannickal
authors.forename: Jerry P
authors.initials: JP
authors.lastname: Eu
mesh_terms: D000818:Animals; D035843:Biomedical Research / Q000639:trends; D004195:Disease Models, Animal; D005109:Extracellular Matrix / Q000473:pathology; D020022:Genetic Predisposition to Disease; D006801:Humans; D054990:Idiopathic Pulmonary Fibrosis / Q000175:diagnosis / Q000503:physiopathology / Q000628:therapy; D007249:Inflammation / Q000276:immunology; D051379:Mice; D011650:Pulmonary Alveoli / Q000473:pathology; D020545:Respiratory Mucosa / Q000473:pathology