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Publications

Journal Article Ahead of print 2 citations

PubMed: 42447235 DOI: 10.1093/ajrccm/aamag363

Acute exacerbation in fibrotic interstitial lung disease: An International Working Group Report.

Am J Respir Crit Care Med

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

Affiliations

  • Respiratory Research@Alfred, School of Translational Medicine, Monash University, Melbourne, Victoria, Australia.
  • Department of Respiratory and Sleep Medicine, Austin Health, Heidelberg, Victoria, Australia.
  • Institute for Breathing and Sleep, Heidelberg, Victoria, Australia.
  • Faculty of Medicine, University of Melbourne, Melbourne, Victoria, Australia.
  • Respiratory Diseases Unit. School of Medicine and Surgery, University of Milano-Bicocca. Fondazione IRCCS "San Gerardo dei Tintori", Monza, Italy.
  • Department of Medicine, Section of Pulmonary & Critical Care, University of Chicago, Chicago, IL USA.
  • Center for Interstitial lung diseases; Division of Pulmonary, Critical Care and Sleep Medicine, Department of Medicine, University of Washington, Seattle, Washington, USA.
  • Department of Medicine, University of California, San Francisco, Division of Pulmonary, Critical Care, Allergy and Sleep Medicine, Division of Clinical Informatics and Digital Transformation, San Francisco, California, USA.
  • Division of Pulmonary and Critical Care Medicine, Massachusetts General Hospital, Boston, MA, USA.
  • Division of Pulmonary and Critical Care Medicine, University of Texas Southwestern Medical Center, Dallas, Texas, USA.
  • National Coordinating Reference Centre for Rare Pulmonary Diseases, OrphaLung, Louis Pradel Hospital, Hospices Civils de Lyon, ERN-LUNG, Lyon, France.
  • UMR 754, Claude Bernard University Lyon 1, INRAE, Lyon, France.
  • Department of Medicine and Snyder Institute for Chronic Diseases, University of Calgary, Calgary AB Canada.
  • Veterans Affairs Center for Innovations in Quality, Effectiveness, and Safety, Michael E DeBakey Veterans Affairs Medical Center, Houston, TX, USA.
  • Section of Pulmonary/Critical Care Medicine and Health Services Research, Department of Medicine, Baylor College of Medicine, USA.
  • Department of Medicine, Firestone Institute for Respiratory Health, McMaster University, Hamilton, ON, Canada.
  • Mainz Center for Pulmonary Medicine, Department of Pneumology, Mainz University Medical Center and Department of Pulmonary, Critical Care and Sleep Medicine, Marienhaus Clinic Mainz, Mainz, Germany.
  • National Heart and Lung Institute, Imperial College London, UK.
  • Royal Brompton and Harefield Hospitals, Guy's and St Thomas' NHS Foundation Trust, London, UK.
  • Centre of Expertise for Interstitial Lung Diseases and Sarcoidosis, Department of Respiratory Medicine, Erasmus MC, Rotterdam, the Netherlands.
  • Dept of Respiratory Medicine, Laboratory of Molecular & Cellular Pneumonology, School of Medicine, University of Crete, Heraklion, Greece.
  • Department of Medicine V, LMU University Hospital, LMU Munich, Comprehensive Pneumology Center, German Center for Lung Research (DZL), Munich, Germany.
  • Centre for Rare Lung Diseases, Department of Respiratory Diseases and Allergy, Aarhus University Hospital, Aarhus, Denmark.
  • Department of Clinical Medicine, Aarhus University, Aarhus, Denmark.
  • Division of Pulmonary and Critical Care Medicine, Department of Medicine, University of California, San Francisco, San Francisco, California, USA.
  • Respiratory and Sleep Medicine, Royal Prince Alfred Hospital, Camperdown, New South Wales, Australia.
  • Faculty of Medicine and Health, University of Sydney, Sydney, New South Wales, Australia.
  • University of Maryland School of Medicine, Baltimore, Maryland, USA.
  • Division of Pulmonary Medicine, Department of Medicine, Faculty of Medicine and Dentistry, Edmonton, AB, Canada.
  • Digital Health Unit, Clinical Trial Office, University of Alberta, AB, Canada.
  • Department of Pathology and Division of Pulmonary and Critical Care Medicine, Massachusetts General Hospital, Harvard Medical School. Boston, MA, USA.
  • Women's Guild Lung Institute, Division of Pulmonary & Critical Care Medicine, Department of Medicine, Cedars-Sinai Medical Center, Los Angeles, CA, USA.
  • Margaret Turner Warwick Centre for Fibrosing Lung Disease, National Heart and Lung Institute, Imperial College London, London, UK.
  • Pulmonary, Critical Care, and Sleep Medicine, Yale School of Medicine, New Haven, Connecticut, USA.
  • Departments of Radiology & Internal Medicine, University of Michigan, Ann Arbor, MI USA.
  • University College Dublin, Dublin, Ireland.
  • Department of Respiratory Medicine and Allergology, Aichi Medical University, Nagakute, Japan.
  • Department of Medicine, Division of Pulmonary, Allergy, and Critical Care Medicine, University of Colorado Anschutz Medical Center, Aurora, Colorado, USA.
  • Department of Pulmonary and Critical Care Medicine, West China Hospital, Sichuan University, Chengdu, China.
  • Keck School of Medicine, University of Southern California, Los Angeles, California, USA.
  • National Heart and Lung Institute, Imperial College London, London, UK.
  • UMass Chan Medical School Boston, Massachusetts, USA.
  • Faculty of Medicine and Health, University of Western Australia, Nedlands, WA, Australia.
  • Department of Respiratory Medicine, Fiona Stanley Hospital, Murdoch, WA, Australia.
  • Fondazione Policlinico Universitario Agostino Gemelli IRCCS Universitas Cattolica del Sacro Cuore Rome, Italy.
  • Rheumatology Unit, Azienda Unità Sanitaria Locale di Piacenza, Piacenza, Italy.
  • Department of Medicine and Surgery, University of Parma, Parma, Italy.
  • Department of Medicine, Virginia Commonwealth University Richmond, Virginia, USA.
  • Global Clinical Development, Boehringer Ingelheim International, Ingelheim am Rhein, Germany.
  • Department of Clinical and Experimental Medicine Careggi University Hospital Florence, Italy.
  • Royal Brompton Hospital and Imperial College, London, UK.
  • Department of Medicine, University of British Columbia, Vancouver, BC, Canada.
  • Centre for Heart Lung Innovation, St. Paul's Hospital, Vancouver, BC, Canada.
  • Department of Medicine, Weill Cornell Medicine, New York, NY, USA.

Abstract

Acute exacerbations (AEs) occur both in patients with idiopathic pulmonary fibrosis (IPF) and 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 AE. 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 DAD 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.

MeSH

Lung, Critical Care, Signs And Symptoms, Pneumonia, Research, Therapeutics, Lung Diseases, Lung Diseases, Interstitial