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Published ahead of print on June 21, 2007, doi:10.1164/rccm.200703-463PP

Am. J. Respir. Crit. Care Med., Volume 176, Number 7, October 2007, 636-643

A more recent version of this article appeared on October 1, 2007
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Submitted on March 21, 2007
Accepted on June 21, 2007

Pulmonary Perspective: Acute Exacerbations of Idiopathic Pulmonary Fibrosis

Harold R Collard1, Bethany B Moore2, Kevin R Flaherty2, Kevin K Brown3, Robert J Kaner4, Talmadge E King, Jr.1, Joseph A Lasky5, James E Lloyd6, Imre Noth7, Mitchell A Olman8, Ganesh Raghu9, Jesse Roman10, Jay H Ryu11, David A Zisman12, Gary W Hunnighake13, Thomas V Colby14, Jim J Egan15, Davd M Hansell16, Takeshi Johkoh17, and Fernando J Martinez2*

1 Department of Medicine, University of California, San Francisco, San Francisco, California, USA, 2 Department of Medicine, University of Michigan, Ann Arbor, Michigan, USA, 3 Department of Medicine, National Jewish Medicial and Research Center, Denver, Colorado, USA, 4 Department of Medicine and Genetic Medicine, Weill Cornell Medical College, New York, New York, USA, 5 Department of Medicine, Tulane University, New Orleans, Louisiana, USA, 6 Department of Medicine, Vanderbilt University, Nashville, Tennessee, USA, 7 Department of Medicine, University of Chicago, Chicago, Illinois, USA, 8 Department of Medicine and Pathology, Universitry of Alabama at Birmingham, Birmingham, Alabama, USA, 9 Department of Medicine, University of Washington, Seattle, Washington, USA, 10 Department of Medicine, Emory University, Atlanta, Georgia, USA, 11 Department of Medicine, Mayo Clinic, Rochester, Minnesota, USA, 12 Department of Medicine, University of California, Los Angeles, California, USA, 13 Department of Medicine, University of Iowa, Iowa City, Iowa, USA, 14 Department of Laboratory Medicine and Pathology, Mayo Clinic, Scottsdale, Arizona, USA, 15 Mater Misericordiae University, Dublin, Ireland, 16 Department of Radiology, Royal Brompton Hospital, London, United Kingdom, 17 Department of Radiology, Osaka University, Osaka, Japan

* To whom correspondence should be addressed. E-mail: fmartine{at}med.umich.edu.

The natural history of idiopathic pulmonary fibrosis (IPF) has been characterized as a steady, predictable decline in lung function over time. Recent evidence suggests that some patients may experience a more precipitous course, with periods of relative stability followed by acute deteriorations in respiratory status. Many of these acute deteriorations are of unknown etiology and have been termed acute exacerbations of IPF. This document is the result of an international effort to summarize the current state of knowledge regarding acute exacerbations of IPF. Acute exacerbations of IPF are defined as acute, clinically-significant deteriorations of unidentifiable cause in patients with underlying IPF. Proposed diagnostic criteria include subjective worsening over 30 days or less, new bilateral radiographic opacities, and the absence of infection or another identifiable etiology. The potential pathobiological roles of infection, disordered cell biology, coagulation, and genetics are discussed, and future research directions are proposed.
Key words: acute exacerbation, idiopathic pulmonary fibrosis, diagnosis




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