The clinical course of patients with idiopathic pulmonary fibrosis.
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.
Martinez
Fernando J
FJ
University of Michigan, Ann Arbor, Michigan 48109, USA. fmartine@med.umich.edu
Safrin
Sharon
S
Weycker
Derek
D
Starko
Karen M
KM
Bradford
Williamson Z
WZ
King
Talmadge E
TE
Flaherty
Kevin R
KR
Schwartz
David A
DA
Noble
Paul W
PW
Raghu
Ganesh
G
Brown
Kevin K
KK
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*