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The Clinical Course of Patients with Idiopathic Pulmonary Fibrosis
- Fernando J. Martinez, MD;
- Sharon Safrin, MD;
- Derek Weycker, PhD;
- Karen M. Starko, MD;
- Williamson Z. Bradford, MD, PhD;
- Talmadge E. King, Jr, MD;
- Kevin R. Flaherty, MD;
- David A. Schwartz, MD;
- Paul W. Noble, MD;
- Ganesh Raghu, MD;
- Kevin K. Brown, MD; and
- for the IPF Study Group*
- From University of Michigan, Ann Arbor, Michigan; InterMune, Inc., Brisbane, California; Policy Analysis, Inc., Brookline, Massachusetts; University of California, San Francisco, San Francisco, California; Duke University School of Medicine, Durham, North Carolina; Yale University, New Haven, Connecticut; University of Washington, Seattle, Washington; and National Jewish Medical and Research Center, Denver, Colorado.
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-γ1b.
Setting: Academic and community medical centers.
Patients: 168 patients in the placebo group of a trial evaluating interferon-γ1b.
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.
*A complete list of the IPF Study Group is available from reference 3: Raghu G, Brown KK, Bradford WZ, Starko K, Noble PW, Schwartz DA, et al. A placebo-controlled trial of interferon gamma-1b in patients with idiopathic pulmonary fibrosis. N Engl J Med. 2004;350:125-33.
Article and Author Information
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Grant Support: By InterMune, Inc.
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Potential Financial Conflicts of Interest: Employment: K.M. Starko (InterMune), W.Z. Bradford (InterMune); Consultancies: F.J. Martinez (InterMune), T.E. King (InterMune), D.A. Schwartz (InterMune), P.W. Noble (InterMune, Genzyme Millenium), G. Raghu (InterMune), K.K. Brown (InterMune, Wyeth, Actelion, Genzyme Corp., FibroGen); Honoraria: T.E. King (InterMune), P.W. Noble (InterMune), G. Raghu (InterMune), K.K. Brown (InteMune, Wyeth, Actelion, Genzyme Corp., FibroGen); Stock ownership or options (other than mutual funds): S. Safrin (InterMune), K.M. Starko (InterMune), W.Z. Bradford (InterMune); Patents pending: K.M. Starko (InterMune), W.Z. Bradford (InterMune).
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Requests for Single Reprints: Fernando Martinez, MD, Department of Internal Medicine, University of Michigan, 3916 Taubman Centers 0360, Ann Arbor, MI 48109; e-mail, fmartine{at}med.umich.edu.
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Current Author Addresses: Dr. Martinez: Department of Internal Medicine, University of Michigan, 3916 Taubman Centers 0360, Ann Arbor, MI 48109.
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Drs. Safrin, Starko, and Bradford: InterMune, Inc., 3280 Bayshore Boulevard, Brisbane, CA 94005.
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Dr. Weycker: Policy Analysis Inc., Four Davis Court, Brookline, MA 02445.
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Dr. King: Department of Medicine, San Francisco General Hospital, 5H22, 1001 Potrero Avenue, San Francisco, CA 94110.
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Dr. Flaherty: University of Michigan Health System, 1500 East Medical Center Drive, 3916 Taubman Center, Ann Arbor, MI 48109.
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Dr. Schwartz: Duke University Medical Center, Box 2629, Research Drive, MSRB Room 275, Durham, NC 27710.
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Dr. Noble: Pulmonary and Critical Care Medicine, Yale School of Medicine, TAC 441-C, 333 Cedar Street, New Haven, CT 06525.
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Dr. Raghu: Division of Pulmonary, University of Washington, BB 1237 Health Sciences, Box 356522, Seattle, WA 98195.
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Dr. Brown: National Jewish Medical and Research Center, 1400 Jackson Street, Denver, CO 80206.
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Author Contributions: Conception and design: F.J. Martinez, S. Safrin, D. Weycker, K.M. Starko, W.Z. Bradford, D.A. Schwartz, P.W. Noble, G. Raghu, K.K. Brown.
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Analysis and interpretation of the data: F.J. Martinez, S. Safrin, D. Weycker, K.M. Starko, W.Z. Bradford, T.E. King Jr., P.W. Noble, G. Raghu, K.K. Brown.
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Drafting of the article: F.J. Martinez, S. Safrin, D. Weycker, K.M. Starko, W.Z. Bradford, D.A. Schwartz, P.W. Noble, K.K. Brown.
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Critical revision of the article for important intellectual content: F.J. Martinez, S. Safrin, D. Weycker, K.M. Starko, T.E. King Jr., K.R. Flaherty, D.A. Schwartz, P.W. Noble, G. Raghu, K.K. Brown.
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Final approval of the article: F.J. Martinez, S. Safrin, D. Weycker, K.M. Starko, T.E. King Jr., K.R. Flaherty, D.A. Schwartz, P.W. Noble, G. Raghu, K.K. Brown.
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Provision of study materials or patients: F.J. Martinez, K.R. Flaherty, D.A. Schwartz, K.K. Brown.
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Statistical expertise: F.J. Martinez, D. Weycker.
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