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

Harold R. Collard(University of California, San Francisco), Christopher J. Ryerson(University of British Columbia), Tamera J. Corte(The University of Sydney), Gísli Jenkins(Nottingham University Hospitals NHS Trust), Yasuhiro Kondoh(Tosei General Hospital), David J. Lederer(Columbia University), Joyce Lee(University of Colorado Denver), Toby M. Maher(Interstitial Cystitis Association), Athol U. Wells(Interstitial Cystitis Association), Κατερίνα Αντωνίου(University of Crete), Jürgen Behr(Asklepios Fachkliniken München-Gauting), Kevin M. Brown, Vincent Cottin(Université Claude Bernard Lyon 1), Kevin R. Flaherty, Junya Fukuoka(Nagasaki University), David M. Hansell(Royal Brompton Hospital), Takeshi Johkoh(Kinki Central Hospital), Naftali Kaminski(Yale University), Dong Soon Kim(University of Ulsan), Martin Kolb(McMaster University), David A. Lynch(National Jewish Health), Jeffrey L. Myers(University of Michigan), Ganesh Raghu(University of Washington), Luca Richeldi(National Institute for Health and Care Research), Hiroyuki Taniguchi(Tosei General Hospital), Fernando J. Martínez(Cornell University)
American Journal of Respiratory and Critical Care Medicine
June 14, 2016
10.1164/rccm.201604-0801ci
Cited by 1,433

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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