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Ann Intern Med. 2005 Jun 21;142(12 Pt 1):963-7.

The clinical course of patients with idiopathic pulmonary fibrosis.

Author information

  • 1University of Michigan, Ann Arbor, Michigan 48109, USA. fmartine@med.umich.edu

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.

PMID:
15968010
[PubMed - indexed for MEDLINE]
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