The clinical course of patients with idiopathic pulmonary fibrosis.

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.

journal_name

Ann Intern Med

authors

Martinez FJ,Safrin S,Weycker D,Starko KM,Bradford WZ,King TE Jr,Flaherty KR,Schwartz DA,Noble PW,Raghu G,Brown KK,IPF Study Group.

doi

10.7326/0003-4819-142-12_part_1-200506210-00005

subject

Has Abstract

pub_date

2005-06-21 00:00:00

pages

963-7

issue

12 Pt 1

eissn

0003-4819

issn

1539-3704

pii

142/12_Part_1/963

journal_volume

142

pub_type

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