title: The clinical course of patients with idiopathic pulmonary fibrosis.
abstract: BACKGROUND
  Prospective data defining the clinical course in idiopathic pulmonary fibrosis (IPF) are sparse.
  
  
  OBJECTIVE
  To analyze the clinical course of patients with mild to moderate IPF.
  
  
  METHODS
  Analysis of data from the placebo group of a randomized, controlled trial evaluating interferon-gamma1b.
  
  
  METHODS
  Academic and community medical centers.
  
  
  METHODS
  168 patients in the placebo group of a trial evaluating interferon-gamma1b.
  
  
  METHODS
  Measures of physiology and dyspnea assessed at 12-week intervals; hospitalizations; and the pace of deterioration and cause of death over a median period of 76 weeks.
  
  
  RESULTS
  Physiologic variables changed minimally during the study. However, 23% of patients required hospitalization for a respiratory disorder and 21% died. Idiopathic pulmonary fibrosis was the primary cause of death in 89% of patients who died, and an apparent acute clinical deterioration preceded death in 47% of these patients.
  
  
  CONCLUSIONS
  The instrument used to define the pace of deterioration and cause of death was applied retrospectively.
  
  
  CONCLUSIONS
  Recognition of the common occurrence of acute fatal deterioration in patients with mild to moderate IPF has important implications for monitoring patients and supports early referral for lung transplantation.
authors.affiliation: University of Michigan, Ann Arbor, Michigan 48109, USA. fmartine@med.umich.edu
authors.forename: Fernando J
authors.initials: FJ
authors.lastname: Martinez
authors.forename: Sharon
authors.initials: S
authors.lastname: Safrin
authors.forename: Derek
authors.initials: D
authors.lastname: Weycker
authors.forename: Karen M
authors.initials: KM
authors.lastname: Starko
authors.forename: Williamson Z
authors.initials: WZ
authors.lastname: Bradford
authors.forename: Talmadge E
authors.initials: TE
authors.lastname: King
authors.forename: Kevin R
authors.initials: KR
authors.lastname: Flaherty
authors.forename: David A
authors.initials: DA
authors.lastname: Schwartz
authors.forename: Paul W
authors.initials: PW
authors.lastname: Noble
authors.forename: Ganesh
authors.initials: G
authors.lastname: Raghu
authors.forename: Kevin K
authors.initials: KK
authors.lastname: Brown
mesh_terms: D000368:Aged; D002423:Cause of Death; D004311:Double-Blind Method; D004417:Dyspnea / Q000209:etiology; D005260:Female; D005500:Follow-Up Studies; D006760:Hospitalization; D006801:Humans; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D011658:Pulmonary Fibrosis / Q000401:mortality / Q000503:physiopathology*