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Clinical Trial
. 2005 Jun 21;142(12 Pt 1):963-7.
doi: 10.7326/0003-4819-142-12_part_1-200506210-00005.

The clinical course of patients with idiopathic pulmonary fibrosis

Fernando J Martinez  1 , Sharon Safrin, Derek Weycker, Karen M Starko, Williamson Z Bradford, Talmadge E King Jr, Kevin R Flaherty, David A Schwartz, Paul W Noble, Ganesh Raghu, Kevin K Brown; IPF Study Group
Affiliations
Clinical Trial

The clinical course of patients with idiopathic pulmonary fibrosis

Fernando J Martinez et al. Ann Intern Med. .

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.

Design: Analysis of data from the placebo group of a randomized, controlled trial evaluating interferon-gamma1b.

Setting: Academic and community medical centers.

Patients: 168 patients in the placebo group of a trial evaluating interferon-gamma1b.

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.

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