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Acute Exacerbations of Idiopathic Pulmonary Fibrosis

¹ Department of Medicine, San Francisco General Hospital, University of California, San Francisco, San Francisco, California; ² Department of Medicine, University of Michigan, Ann Arbor, Michigan; ³ Department of Medicine, National Jewish Medical and Research Center, Denver, Colorado; ⁴ Department of Medicine and Genetic Medicine, Weill Cornell Medical College, New York, New York; ⁵ Department of Medicine, Tulane University, New Orleans, Louisiana; ⁶ Department of Medicine, Vanderbilt University, Nashville, Tennessee; ⁷ Department of Medicine, University of Chicago, Chicago, Illinois; ⁸ Department of Medicine and Pathology, University of Alabama at Birmingham, Birmingham, Alabama; ⁹ Department of Medicine, University of Washington, Seattle, Washington; ¹⁰ Department of Medicine, Emory University, Atlanta, Georgia; ¹¹ Department of Medicine, Mayo Clinic, Rochester, Minnesota; ¹² Department of Medicine, University of California Los Angeles, Los Angeles, California; ¹³ Department of Medicine, University of Iowa, Iowa City, Iowa; ¹⁴ Department of Laboratory Medicine and Pathology, Mayo Clinic Scottsdale, Scottsdale, Arizona; ¹⁵ Mater Misericordiae University Hospital, University College, Dublin, Ireland; ¹⁶ Department of Radiology, Royal Brompton Hospital, Imperial College London, London, United Kingdom; ¹⁷ Department of Radiology, Osaka University, Osaka, Japan; ¹⁸ Department of Medicine, University of Pittsburgh, Pittsburgh, Pennsylvania; ¹⁹ Division of Pulmonary and Critical Care Medicine, Asan Medical Center, University of Ulsan, Seoul, South Korea; ²⁰ Department of Respiratory Medicine and Allergy, Tosei General Hospital, Aichi, Japan; ²¹ Department of Radiology, National Jewish Medical and Research Center, Denver, Colorado; ²² Department of Pneumology, University Medical Center Freiburg, Freiburg, Germany; ²³ Department of Pathology, University of Michigan, Ann Arbor, Michigan; ²⁴ Department of Histopathology, Royal Brompton Hospital, Imperial College London, London, United Kingdom; ²⁵ Instituto Nacional de Enfermedades Respiratorias, Mexico City, Mexico; and ²⁶ Department of Respiratory Medicine, Royal Brompton Hospital, Imperial College London, London, United Kingdom

Correspondence and requests for reprints should be addressed to Fernando J. Martinez, M.D., University of Michigan Health System, 1500 East Medical Center Drive, 3916 Taubman Center, Ann Arbor, MI 48109-0360. E-mail: fmartine{at}med.umich.edu

ABSTRACT

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 perspective 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 • pulmonary fibrosis • diagnosis • definition

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