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Am J Respir Crit Care Med. 2016 Aug 1;194(3):265-75. doi: 10.1164/rccm.201604-0801CI.

Acute Exacerbation of Idiopathic Pulmonary Fibrosis. An International Working Group Report.

Author information

1
1 Department of Medicine, University of California San Francisco, San Francisco, California.
2
2 Department of Medicine, University of British Columbia, Vancouver, British Columbia, Canada.
3
3 Department of Respiratory Medicine, Royal Prince Alfred Hospital and University of Sydney, Sydney, Australia.
4
4 Division of Respiratory Medicine, Nottingham University Hospitals, Nottingham, United Kingdom.
5
5 Department of Respiratory Medicine and Allergy, Tosei General Hospital, Aichi, Japan.
6
6 Department of Medicine, Columbia University, New York, New York.
7
7 Department of Medicine, University of Colorado Denver, Aurora, Colorado.
8
8 National Heart and Lung Institute, Imperial College London, London, United Kingdom.
9
9 Interstitial Lung Disease Unit and.
10
10 Department of Respiratory Medicine, Faculty of Medicine, University of Crete, Heraklion, Greece.
11
11 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.
12
12 Department of Medicine and.
13
13 Department of Medicine, Louis Pradel Hospital, Hospices Civils de Lyon, Claude Bernard University Lyon 1, University of Lyon, Lyon, France.
14
14 Department of Medicine and.
15
15 Department of Pathology, Nagasaki University, Nagasaki, Japan.
16
16 Department of Radiology, Royal Brompton Hospital, London, United Kingdom.
17
17 Department of Radiology, Kinki Central Hospital, Itami, Japan.
18
18 Pulmonary, Critical Care, and Sleep Medicine, Department of Medicine, Yale School of Medicine, New Haven, Connecticut.
19
19 Department of Medicine, University of Ulsan, Seoul, South Korea.
20
20 Department of Medicine, McMaster University, Hamilton, Ontario, Canada.
21
21 Department of Radiology, National Jewish Health, Denver, Colorado.
22
22 Department of Pathology, University of Michigan, Ann Arbor, Michigan.
23
23 Department of Medicine, University of Washington, Seattle, Washington.
24
24 Academic Unit of Clinical and Experimental Sciences, University of Southampton and National Institute for Health Research Southampton Respiratory Biomedical Unit, Southampton, United Kingdom; and.
25
25 Department of Medicine, Weill Cornell University, New York, New York.

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.

KEYWORDS:

acute exacerbation; diagnosis; management; pulmonary fibrosis; review

PMID:
27299520
DOI:
10.1164/rccm.201604-0801CI
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