This Article Full Text Full Text (PDF) All Versions of this Article: 200703-463PPv1 176/7/636    most recent Alert me when this article is cited Alert me if a correction is posted Services Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Collard, H. R. Search for Related Content PubMed PubMed Citation Articles by Collard, H. R.

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

This article has been cited by other articles:

				A. M. Tager, A. Sharma, and E. J. Mark Case 32-2009 -- A 27-Year-Old Man with Progressive Dyspnea N. Engl. J. Med., October 15, 2009; 361(16): 1585 - 1593. [Full Text] [PDF]

				K. Konishi, K. F. Gibson, K. O. Lindell, T. J. Richards, Y. Zhang, R. Dhir, M. Bisceglia, S. Gilbert, S. A. Yousem, J. W. Song, et al. Gene Expression Profiles of Acute Exacerbations of Idiopathic Pulmonary Fibrosis Am. J. Respir. Crit. Care Med., July 15, 2009; 180(2): 167 - 175. [Abstract] [Full Text] [PDF]

				A. L. Olson, J. J. Swigris, G. Raghu, and K. K. Brown Seasonal Variation: Mortality From Pulmonary Fibrosis Is Greatest in the Winter Chest, July 1, 2009; 136(1): 16 - 22. [Abstract] [Full Text] [PDF]

				K. Sakamoto, H. Taniguchi, Y. Kondoh, K. Ono, Y. Hasegawa, and M. Kitaichi Acute exacerbation of idiopathic pulmonary fibrosis as the initial presentation of the disease Eur. Respir. Rev., June 1, 2009; 18(112): 129 - 132. [Abstract] [Full Text] [PDF]

				A. Prasse, C. Probst, E. Bargagli, G. Zissel, G. B. Toews, K. R. Flaherty, M. Olschewski, P. Rottoli, and J. Muller-Quernheim Serum CC-Chemokine Ligand 18 Concentration Predicts Outcome in Idiopathic Pulmonary Fibrosis Am. J. Respir. Crit. Care Med., April 15, 2009; 179(8): 717 - 723. [Abstract] [Full Text] [PDF]

				A. Moeller, S. E. Gilpin, K. Ask, G. Cox, D. Cook, J. Gauldie, P. J. Margetts, L. Farkas, J. Dobranowski, C. Boylan, et al. Circulating Fibrocytes Are an Indicator of Poor Prognosis in Idiopathic Pulmonary Fibrosis Am. J. Respir. Crit. Care Med., April 1, 2009; 179(7): 588 - 594. [Abstract] [Full Text] [PDF]

				M. Kreider and R. M. Kotloff Selection of Candidates for Lung Transplantation Proceedings of the ATS, January 15, 2009; 6(1): 20 - 27. [Abstract] [Full Text] [PDF]

				J. P. Lynch III Idiopathic Pulmonary Fibrosis, Nonspecific Interstitial Pneumonia/Fibrosis, and Sarcoidosis ACCP Pulmonary Med Brd Rev, January 1, 2009; 25(0): 635 - 686. [Full Text] [PDF]

				T. E. King Jr and R. M. du Bois Elucidating the causes and examining the latest clinical findings in pulmonary fibrosis Eur. Respir. Rev., December 1, 2008; 17(109): 105 - 107. [Full Text] [PDF]

				T M Maher and A U Wells Optimal treatment for idiopathic pulmonary fibrosis Thorax, December 1, 2008; 63(12): 1120 - 1121. [Full Text] [PDF]

				G. Raghu, K. K. Brown, U. Costabel, V. Cottin, R. M. du Bois, J. A. Lasky, M. Thomeer, J. P. Utz, R. K. Khandker, L. McDermott, et al. Treatment of Idiopathic Pulmonary Fibrosis with Etanercept: An Exploratory, Placebo-controlled Trial Am. J. Respir. Crit. Care Med., November 1, 2008; 178(9): 948 - 955. [Abstract] [Full Text] [PDF]

				H. Arakawa, K. Fujimoto, K. Honma, N. Suganuma, H. Morikubo, Y. Saito, H. Shida, and Y. Kaji Progression from Near-Normal to End-Stage Lungs in Chronic Interstitial Pneumonia Related to Silica Exposure: Long-Term CT Observations Am. J. Roentgenol., October 1, 2008; 191(4): 1040 - 1045. [Abstract] [Full Text] [PDF]

				A U Wells, N Hirani, and on behalf of the BTS Interstitial Lung Disease Gui Interstitial lung disease guideline Thorax, September 1, 2008; 63(Suppl_5): v1 - v58. [Full Text] [PDF]

				M. Akira, T. Kozuka, S. Yamamoto, and M. Sakatani Computed Tomography Findings in Acute Exacerbation of Idiopathic Pulmonary Fibrosis Am. J. Respir. Crit. Care Med., August 15, 2008; 178(4): 372 - 378. [Abstract] [Full Text] [PDF]

				F. R. Khuri and J. Roman From Nihilism to Individualism: The Evolution of Lung Cancer Therapy Am. J. Respir. Crit. Care Med., June 15, 2008; 177(12): 1299 - 1300. [Full Text] [PDF]

				W. A. Wuyts, M. Thomeer, L. J. Dupont, and G. M. Verleden An Algorithm to Tackle Acute Exacerbations in Idiopathic Pulmonary Fibrosis Am. J. Respir. Crit. Care Med., June 15, 2008; 177(12): 1397 - 1397. [Full Text] [PDF]

				H. R. Collard and F. J. Martinez Basis of Acute Exacerbation of Idiopathic Pulmonary Fibrosis in Japanese Patients Am. J. Respir. Crit. Care Med., June 15, 2008; 177(12): 1398 - 1398. [Full Text] [PDF]

				A. Azuma, K. Hagiwara, and S. Kudoh Basis of Acute Exacerbation of Idiopathic Pulmonary Fibrosis in Japanese Patients Am. J. Respir. Crit. Care Med., June 15, 2008; 177(12): 1397 - 1398. [Full Text] [PDF]

				G. Trujillo, E. C. O'Connor, S. L. Kunkel, and C. M. Hogaboam A Novel Mechanism for CCR4 in the Regulation of Macrophage Activation in Bleomycin-Induced Pulmonary Fibrosis Am. J. Pathol., May 1, 2008; 172(5): 1209 - 1221. [Abstract] [Full Text] [PDF]

				P. Rogliani, M. Mura, M. Assunta Porretta, and C. Saltini Review: New perspectives in the treatment of idiopathic pulmonary fibrosis Therapeutic Advances in Respiratory Disease, April 1, 2008; 2(2): 75 - 93. [Abstract] [PDF]

				T. R. McMillan, B. B. Moore, J. B. Weinberg, K. M. Vannella, W. B. Fields, P. J. Christensen, L. F. van Dyk, and G. B. Toews Exacerbation of Established Pulmonary Fibrosis in a Murine Model by Gammaherpesvirus Am. J. Respir. Crit. Care Med., April 1, 2008; 177(7): 771 - 780. [Abstract] [Full Text] [PDF]

				A. U. Wells and C. M. Hogaboam Update in Diffuse Parenchymal Lung Disease 2007 Am. J. Respir. Crit. Care Med., March 15, 2008; 177(6): 580 - 584. [Full Text] [PDF]

				K. R. Flaherty High-Resolution Computed Tomography and the Many Faces of Idiopathic Pulmonary Fibrosis Am. J. Respir. Crit. Care Med., February 15, 2008; 177(4): 367 - 368. [Full Text] [PDF]

				B. B. Moore and C. M. Hogaboam Murine models of pulmonary fibrosis Am J Physiol Lung Cell Mol Physiol, February 1, 2008; 294(2): L152 - L160. [Abstract] [Full Text] [PDF]