Related Experiment Video
Updated: Jul 16, 2026
Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
Published on: June 16, 2020
The clinical course of patients with idiopathic pulmonary fibrosis
Fernando J Martinez1, Sharon Safrin, Derek Weycker
1University of Michigan, Ann Arbor, Michigan 48109, USA. fmartine@med.umich.edu
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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