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Abstract: Slide Presentations |

Rates and Characteristics of Death in Patients With Idiopathic Pulmonary Fibrosis (IPF) FREE TO VIEW

Fernando I. Martinez, MD; Williamson Z. Bradford, MD; Sharon Safrin, MD; Karen M. Starko, MD; Kevin K. Brown, MD; IPF Study Group
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University of Michigan, Ann Arbor, IL


Chest


Chest. 2003;124(4_MeetingAbstracts):117S. doi:10.1378/chest.124.4_MeetingAbstracts.117S-b
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Abstract

PURPOSE:  A randomized, double-blind, placebo-controlled study evaluating IFN-γ 1b for treatment of steroid-refractory IPF was performed in 330 patients. Primary results were reported previously. To further characterize the natural history of IPF, a detailed questionnaire was sent to all sites at which a study patient died during the study.

METHODS:  Investigators were queried as to the primary cause of death, whether the death was respiratory in nature and/or related to IPF, and the time from terminal event to death.

RESULTS:  In this well-defined, highly protocol-adherent patient population, 62 patients died during a median follow-up of 72 weeks: 26/162 (16%) IFN-γ 1b vs. 36/168 (21%) placebo. Progression of IPF was considered the primary cause of death in 53% (13 IFN-γ 1b vs. 20 placebo patients), pneumonia in 15% (5 vs. 4), cardiac or cerebral ischemic event in 5% (1 vs. 2), ARDS in 3% (0 vs. 2), and miscellaneous or unknown causes in the remainder. Death was described as related to IPF in 85% (21 vs. 32 patients). In the 53 patients who died of an IPF-related cause, the terminal event was abrupt or acute (i.e., ≥ 4 wks) in 47% (10 IFN-γ 1b vs. 15 placebo patients) and progressive (i.e, wks – mos) in 49% (10 vs. 16 patients). Comparison of the last measures available prior to death with baseline values showed more marked deterioration in mean % predicted FVC, A-a gradient, and dyspnea score in patients who died vs. survivors.CONCLUSIONS: In patients with steroid-refractory IPF, > 50% die of disease progression. In patients dying of an IPF-related cause, abrupt or acute decompensation and progressive deterioration occurred with nearly equal frequency.

CLINICAL IMPLICATIONS:  The natural history of IPF bears further investigation. Death due to acute decompensation may have different mechanisms and therapeutic responsiveness than that due to progressive deterioration.

DISCLOSURE:  F.I. Martinez, InterMune, Inc., Grant monies.

Tuesday, October 28, 2003

2:30 PM - 4:00 PM


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