Acute Exacerbation of Idiopathic Pulmonary Fibrosis. An International Working Group Report
Abstract
Acute exacerbation of idiopathic pulmonary fibrosis has been defined as an acute, clinically significant, respiratory deterioration of unidentifiable cause. The objective of this international working group report on acute exacerbation of idiopathic pulmonary fibrosis was to provide a comprehensive update on the topic. A literature review was conducted to identify all relevant English text publications and abstracts. Evidence-based updates on the epidemiology, etiology, risk factors, prognosis, and management of acute exacerbations of idiopathic pulmonary fibrosis are provided. Finally, to better reflect the current state of knowledge and improve the feasibility of future research into its etiology and treatment, the working group proposes a new conceptual framework for acute respiratory deterioration in idiopathic pulmonary fibrosis and a revised definition and diagnostic criteria for acute exacerbation of idiopathic pulmonary fibrosis.
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An unintended consequence of rentosertib's potential immunomodulatory mechanism of action may be a dampened site-specific immune response, potentially leading to increased susceptibility to infection, a known trigger for a subset of AE-IPF.
“An unintended consequence of rentosertib’s potential immunomodulatory mechanism of action9 may be a dampened site-specific immune response, potentially leading to increased susceptibility to infection, a known trigger for a subset of AE-IPF45,46.”
From A generative AI-discovered TNIK inhibitor for idiopathic pulmonary fibrosis: a randomized phase 2a trial (2025) · cites this paper as [46]
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- Authors
- Collard, H. R., Ryerson, C. J., Corte, T. J., Jenkins, G., Kondoh, Y., Lederer, D. J., Lee, J. S., Maher, T. M., Wells, A. U., Antoniou, K. M., Behr, J., Brown, K. K., Cottin, V., Flaherty, K. R., Fukuoka, J., Hansell, D. M., Johkoh, T., Kaminski, N., Kim, D. S., Kolb, M., Lynch, D. A., Myers, J. L., Raghu, G., Richeldi, L., Taniguchi, H., & Martinez, F. J.
- Affiliations
- University of California, San Francisco (US)
- University of British Columbia (CA)
- The University of Sydney (AU)
- Nottingham University Hospitals NHS Trust (GB)
- Tosei General Hospital (JP)
- Columbia University (US)
- University of Colorado Denver (US)
- Imperial College London (GB)
- Interstitial Lung Disease Unit and
- University of Crete (GR)
- Department of Internal Medicine V, Ludwig Maximilians University, Munich, and Asklepios Clinics, Gauting, Comprehensive Pneumology Center Munich, Member of the German Center for Lung Research, Germany
- Department of Medicine and
- Lyon 1 Université (FR)
- Nagasaki University (JP)
- Royal Brompton Hospital (GB)
- Kinki Central Hospital (JP)
- Yale University (US)
- University of Ulsan (KR)
- McMaster University (CA)
- National Jewish Health (US)
- University of Michigan (US)
- University of Washington (US)
- National Institute for Health and Care Research (GB)
- Department of Medicine, Weill Cornell University, New York, New York
- Corresponding author
- Collard, Harold R.
- ORCID
- Collard, Harold R.
- Ryerson, Christopher J.
- Corte, Tamera J.
- Jenkins, Gisli
- Kondoh, Yasuhiro
- Lederer, David J.
- Lee, Joyce S.
- Maher, Toby M.
- Wells, Athol U.
- Antoniou, Katerina M.
- Cottin, Vincent
- Flaherty, Kevin R.
- Fukuoka, Junya
- Johkoh, Takeshi
- Kaminski, Naftali
- Kolb, Martin
- Lynch, David A.
- Myers, Jeffrey L.
- Raghu, Ganesh
- Richeldi, Luca
- Taniguchi, Hiroyuki
- Venue
- American Journal of Respiratory and Critical Care Medicine
- Publisher
- Oxford University Press (OUP)
- Published
- 2016-08-01
- Volume
- 194
- Issue
- 3
- Pages
- 265-275
- ISSN
- 1073-449X (ISSN-L)
- 1535-4970
- OpenAlex
- W2440054067
- Cited by
- 1,444
- References
- 6
- Citations by year
- 2026: 109 · 2025: 133 · 2024: 169 · 2023: 155 · 2022: 172 · 2021: 188 · 2020: 153 · 2019: 157 · 2018: 103 · 2017: 90 · 2016: 12 · 2015: 1
- FWCI
- 20.89
- Type
- journal article
- Language
- en
- Open access
- closed
- Locations
- American Journal of Respiratory and Critical Care Medicine (publishedVersion)
- PubMed (publishedVersion)
- Spiral (Imperial College London) (submittedVersion)
- Topics
- Health Sciences › Medicine › Pulmonary and Respiratory Medicine › Interstitial Lung Diseases and Idiopathic Pulmonary Fibrosis
- Health Sciences › Medicine › Pulmonary and Respiratory Medicine › Chronic Obstructive Pulmonary Disease (COPD) Research
- Health Sciences › Medicine › Pulmonary and Respiratory Medicine › Occupational and environmental lung diseases
- Keywords
- idiopathic pulmonary fibrosis, acute exacerbation, etiology, risk factors, prognosis, conceptual framework
- MeSH terms
- Acute Disease, Humans, Prognosis, Risk Factors, Disease Management, Internationality, Idiopathic Pulmonary Fibrosis
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