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.

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.