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-gamma 1b. Setting: Academic and community medical centers. Patients: 168 patients in the placebo group of a trial evaluating interferon-gamma 1b. 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.