title: Acute exacerbations of idiopathic pulmonary fibrosis.
abstract: The natural history of idiopathic pulmonary fibrosis (IPF) has been characterized as a steady, predictable decline in lung function over time. Recent evidence suggests that some patients may experience a more precipitous course, with periods of relative stability followed by acute deteriorations in respiratory status. Many of these acute deteriorations are of unknown etiology and have been termed acute exacerbations of IPF. This perspective is the result of an international effort to summarize the current state of knowledge regarding acute exacerbations of IPF. Acute exacerbations of IPF are defined as acute, clinically significant deteriorations of unidentifiable cause in patients with underlying IPF. Proposed diagnostic criteria include subjective worsening over 30 days or less, new bilateral radiographic opacities, and the absence of infection or another identifiable etiology. The potential pathobiological roles of infection, disordered cell biology, coagulation, and genetics are discussed, and future research directions are proposed.
authors.affiliation: Department of Medicine, San Francisco General Hospital, University of California, USA.
authors.forename: Harold R
authors.initials: HR
authors.lastname: Collard
authors.forename: Bethany B
authors.initials: BB
authors.lastname: Moore
authors.forename: Kevin R
authors.initials: KR
authors.lastname: Flaherty
authors.forename: Kevin K
authors.initials: KK
authors.lastname: Brown
authors.forename: Robert J
authors.initials: RJ
authors.lastname: Kaner
authors.forename: Talmadge E
authors.initials: TE
authors.lastname: King
authors.forename: Joseph A
authors.initials: JA
authors.lastname: Lasky
authors.forename: James E
authors.initials: JE
authors.lastname: Loyd
authors.forename: Imre
authors.initials: I
authors.lastname: Noth
authors.forename: Mitchell A
authors.initials: MA
authors.lastname: Olman
authors.forename: Ganesh
authors.initials: G
authors.lastname: Raghu
authors.forename: Jesse
authors.initials: J
authors.lastname: Roman
authors.forename: Jay H
authors.initials: JH
authors.lastname: Ryu
authors.forename: David A
authors.initials: DA
authors.lastname: Zisman
authors.forename: Gary W
authors.initials: GW
authors.lastname: Hunninghake
authors.forename: Thomas V
authors.initials: TV
authors.lastname: Colby
authors.forename: Jim J
authors.initials: JJ
authors.lastname: Egan
authors.forename: David M
authors.initials: DM
authors.lastname: Hansell
authors.forename: Takeshi
authors.initials: T
authors.lastname: Johkoh
authors.forename: Naftali
authors.initials: N
authors.lastname: Kaminski
authors.forename: Dong Soon
authors.initials: DS
authors.lastname: Kim
authors.forename: Yasuhiro
authors.initials: Y
authors.lastname: Kondoh
authors.forename: David A
authors.initials: DA
authors.lastname: Lynch
authors.forename: Joachim
authors.initials: J
authors.lastname: Müller-Quernheim
authors.forename: Jeffrey L
authors.initials: JL
authors.lastname: Myers
authors.forename: Andrew G
authors.initials: AG
authors.lastname: Nicholson
authors.forename: Moisés
authors.initials: M
authors.lastname: Selman
authors.forename: Galen B
authors.initials: GB
authors.lastname: Toews
authors.forename: Athol U
authors.initials: AU
authors.lastname: Wells
authors.forename: Fernando J
authors.initials: FJ
authors.lastname: Martinez
mesh_terms: D000208:Acute Disease; D006801:Humans; D008168:Lung; D011658:Pulmonary Fibrosis / Q000175:diagnosis / Q000209:etiology / Q000473:pathology / Q000503:physiopathology; D012307:Risk Factors