The clinical course of patients with idiopathic pulmonary fibrosis
FJ Martinez, S Safrin, D Weycker, KM Starko… - Annals of internal …, 2005 - acpjournals.org
FJ Martinez, S Safrin, D Weycker, KM Starko, WZ Bradford, TE King Jr, KR Flaherty…
Annals of internal medicine, 2005•acpjournals.orgBackground: 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. Design: Analysis of data from the placebo group of a randomized, controlled trial
evaluating interferon-γ1b. Setting: Academic and community medical centers. Patients: 168
patients in the placebo group of a trial evaluating interferon-γ1b. Measurements: Measures
of physiology and dyspnea assessed at 12-week intervals; hospitalizations; and the pace of …
(IPF) are sparse. Objective: To analyze the clinical course of patients with mild to moderate
IPF. Design: Analysis of data from the placebo group of a randomized, controlled trial
evaluating interferon-γ1b. Setting: Academic and community medical centers. Patients: 168
patients in the placebo group of a trial evaluating interferon-γ1b. Measurements: Measures
of physiology and dyspnea assessed at 12-week intervals; hospitalizations; and the pace of …
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.
Design
Analysis of data from the placebo group of a randomized, controlled trial evaluating interferon-γ1b.
Setting
Academic and community medical centers.
Patients
168 patients in the placebo group of a trial evaluating interferon-γ1b.
Measurements
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.
Limitations
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.
*A complete list of the IPF Study Group is available from reference : Raghu G, Brown KK, Bradford WZ, Starko K, Noble PW, Schwartz DA, et al. A placebo-controlled trial of interferon gamma-1b in patients with idiopathic pulmonary fibrosis. N Engl J Med. 2004;350:125-33.
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