Elisabeth Bendstrup

Acute exacerbation of idiopathic pulmonary fibrosis: International survey and call for harmonisation

Research output: Contribution to journal/Conference contribution in journal/Contribution to newspaperJournal articleResearchpeer-review

  • Michael Kreuter, Heidelberg University , German Center for Lung Research (DZL)
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  • Markus Polke, Heidelberg University 
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  • Simon L.F. Walsh, National Heart and Lung Institute
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  • Johannes Krisam, Heidelberg University 
  • ,
  • Harold R. Collard, University of California at San Francisco
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  • Nazia Chaudhuri, Manchester University NHS Foundation Trust
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  • Sergey Avdeev, I.M. Sechenov First Moscow State Medical University of the Ministry of Healthcare of the Russian Federation (Sechenovskiy University)
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  • Jürgen Behr, Ludwig-Maximilians-University München, German Center for Lung Research
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  • Gregory Calligaro, University of Cape Town
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  • Tamera Corte, Royal Prince Alfred Hospital
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  • Kevin Flaherty, University of Michigan, Ann Arbor, Michigan.
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  • Manuela Funke-Chambour, University of Bern
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  • Martin Kolb, McMaster University
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  • Yasuhiro Kondoh, Tosei General Hospital
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  • Toby M. Maher, National Heart and Lung Institute, The National Institute for Health Research Collaboration for Leadership in Applied Health Research and Care West (NIHR CLAHRC West) at University Hospitals Bristol NHS Foundation Trust
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  • Maria Molina Molina, Hospital Universitari de Bellvitge, CIBER de Enfermedades Respiratorias (CIBERES)
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  • Antonio Morais, University of Porto
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  • Catharina C. Moor, Erasmus University Rotterdam
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  • Julie Morisset, Université de Montréal
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  • Carlos Pereira, Universidade Federal de Sao Paulo
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  • Silvia Quadrelli, Hospital Británico, Sanatorio Güemes
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  • Moises Selman, National Institute of Respiratory Diseases
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  • Argyrios Tzouvelekis, University of Athens
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  • Claudia Valenzuela, Hospital Universitario de la Princesa
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  • Carlo Vancheri, University of Catania
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  • Vanesa Vicens-Zygmunt, Bellvitge Biomedical Research Institute, Hospital Universitari de Bellvitge
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  • Julia Wälscher, Heidelberg University 
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  • Wim Wuyts, Dentistry, University Hospitals, KU Leuven
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  • Marlies Wijsenbeek, Erasmus University Rotterdam
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  • Vincent Cottin, Hopital Louis Pradel, Claude Bernard University Lyon 1
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  • Elisabeth Bendstrup

Acute exacerbation of idiopathic pulmonary fibrosis (AE-IPF) is an often deadly complication of IPF. No focussed international guidelines for the management of AE-IPF exist. The aim of this international survey was to assess the global variability in prevention, diagnostic and treatment strategies for AE-IPF. Pulmonologists with ILD expertise were invited to participate in a survey designed by an international expert panel. 509 pulmonologists from 66 countries responded. Significant geographical variability in approaches to manage AE-IPF was found. Common preventive measures included antifibrotic drugs and vaccination. Diagnostic differences were most pronounced regarding use of Krebs von den Lungen-6 and viral testing, while high-resolution computed tomography, brain natriuretic peptide and D-dimer are generally applied. High-dose steroids are widely administered (94%); the use of other immunosuppressant and treatment strategies is highly variable. Very few (4%) responders never use immunosuppression. Antifibrotic treatments are initiated during AE-IPF by 67%. Invasive ventilation or extracorporeal membrane oxygenation are mainly used as a bridge to transplantation. Most physicians educate patients comprehensively on the severity of AE-IPF (82%) and consider palliative care (64%). Approaches to the prevention, diagnosis and treatment of AE-IPF vary worldwide. Global trials and guidelines to improve the prognosis of AE-IPF are needed.

Original languageEnglish
Article number1901760
Book seriesEuropean Respiratory Journal
Volume55
Issue4
Number of pages9
ISSN0903-1936
DOIs
Publication statusPublished - 2020

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