Acute exacerbation in fibrotic interstitial lung disease: an international working group report
Year: 2026
Type: review
Abstract: Acute exacerbations (AEs) occur both in patients with idiopathic pulmonary fibrosis (IPF) and those with non-IPF fibrotic interstitial lung disease (fILD). These events confer high morbidity and mortality, with a lack of proven effective therapeutic interventions. The objective of this state-of-the-art document is to summarize latest evidence since the 2016 International Working Group Report on AE-IPF, expanding it across the spectrum of all fILDs. A comprehensive literature review on the epidemiology, associated and risk factors, prognosis, and management of AE-fILD is summarized. In addition to revising the AE definition and diagnostic criteria for broad application across different fILDs, a conceptual framework for acute respiratory worsening (ARW) has been proposed to encompass a variety of acute respiratory deteriorations, both related and unrelated to AEs. This allows structured evaluation in both clinical and research settings. The proposed revised definition for AE-fILD is an acute respiratory event characterized by increased respiratory symptoms or signs and associated with radiologic or histologic features consistent with diffuse alveolar damage (with or without superimposed organizing pneumonia) in a patient with known or newly diagnosed fILD. On the other hand, ARW refers to a heterogeneous group of clinical events with acute symptom worsening not attributable to diffuse alveolar damage in patients with fILD, such as pulmonary edema, bronchitis, and pneumonia, although severe pneumonia can trigger AE-fILD. Additionally, we discuss considerations for inclusion of AE as a clinical trial endpoint, as well as research priorities for advancing knowledge on the pathogenic mechanisms, event prediction, risk stratification, and development of drugs and supportive treatments.
Authors: Yet H Khor, Fabrizio Luppi, Ayodeji Adegunsoye, Bridget F Collins, Erica Farrand, Sydney B Montesi, Chad A Newton, Vincent Cottin, Kerri A Johannson, Bhavika Kaul, Martin Kolb, Michael Kreuter, Philip L Molyneaux, Marlies S Wijsenbeek, Katerina Antoniou, Jürgen Behr, Elisabeth Bendstrup, Harold R Collard, Tamera J Corte, Wonder P Drake, Giovanni Ferrara, Lida P Hariri, Cory M Hogaboam, R Gisli Jenkins, Naftali Kaminski, Ella A Kazerooni, Michael P Keane, Yasuhiro Kondoh, Joyce S Lee, Fengming Luo, Toby M Maher, Fernando J Martinez, Yuben Moodley, Luca Richeldi, Marco Sebastiani, Patricia J Sime, Susanne Stowasser, Sara Tomassetti, Athol Wells, Christopher J Ryerson, Anna J Podolanczuk
Institutions: The University of Melbourne, Institute for Breathing and Sleep, Austin Health, Monash University, University of Chicago, University of Washington, University of California, San Francisco, Massachusetts General Hospital, The University of Texas Southwestern Medical Center, Lyon 1 Université, Institut National de Recherche pour l'Agriculture, l'Alimentation et l'Environnement, Hospices Civils de Lyon, Hôpital Louis Pradel, Infections Virales et Pathologie Comparée, University of Calgary, Michael E. DeBakey VA Medical Center, Baylor College of Medicine, McMaster University, University Medical Center of the Johannes Gutenberg University Mainz, Guy's and St Thomas' NHS Foundation Trust, Harefield Hospital, Imperial College London, Erasmus MC, University of Crete, LMU Klinikum, German Center for Lung Research, Ludwig-Maximilians-Universität München, Aarhus University, Aarhus University Hospital, The University of Sydney, Royal Prince Alfred Hospital, University of Maryland, Baltimore, Alberta Health Services, University of Alberta, Harvard University, Cedars-Sinai Medical Center, Yale University, University of Michigan, Michigan Medicine, University College Dublin, Aichi Medical University, Aichi Medical University Hospital, University of Colorado Anschutz, Sichuan University, West China Hospital of Sichuan University, University of Southern California, University of Massachusetts Chan Medical School, The University of Western Australia, Fiona Stanley Hospital, Università Cattolica del Sacro Cuore, Agostino Gemelli University Polyclinic, Istituti di Ricovero e Cura a Carattere Scientifico, University of Parma, Azienda Unità Sanitaria Locale Piacenza, Virginia Commonwealth University, Boehringer Ingelheim (Germany), Azienda Ospedaliero-Universitaria Careggi, National Health Service, Royal Brompton Hospital, University of British Columbia, St. Paul's Hospital, Centre for Heart Lung Innovation, Weill Cornell Medicine
Language: English
Subfield: Pulmonary and Respiratory Medicine
Field: Medicine
Domain: Health Sciences
Open Access status: hybrid
OpenAlex ID: W7168265538