Management of Acute Exacerbation of Idiopathic Pulmonary Fibrosis in Specialised and Non-specialised ILD Centres Around the World

Markus Polke, Yasuhiro Kondoh, Marlies Wijsenbeek, Vincent Cottin, Simon L F Walsh, Harold R Collard, Nazia Chaudhuri, Sergey Avdeev, Jürgen Behr, Gregory Calligaro, Tamera J Corte, Kevin Flaherty, Manuela Funke-Chambour, Martin Kolb, Johannes Krisam, Toby M Maher, Maria Molina Molina, Antonio Morais, Catharina C Moor, Julie MorissetCarlos Pereira, Silvia Quadrelli, Moises Selman, Argyrios Tzouvelekis, Claudia Valenzuela, Carlo Vancheri, Vanesa Vicens-Zygmunt, Julia Wälscher, Wim Wuyts, Elisabeth Bendstrup, Michael Kreuter

Research output: Contribution to journalArticlepeer-review

7 Citations (Scopus)
9 Downloads (Pure)


Background: Acute exacerbation of idiopathic pulmonary fibrosis (AE-IPF) is a severe complication associated with a high mortality. However, evidence and guidance on management is sparse. The aim of this international survey was to assess differences in prevention, diagnostic and treatment strategies for AE-IPF in specialised and non-specialised ILD centres worldwide. Material and Methods: Pulmonologists working in specialised and non-specialised ILD centres were invited to participate in a survey designed by an international expert panel. Responses were evaluated in respect to the physicians' institutions. Results: Three hundred and two (65%) of the respondents worked in a specialised ILD centre, 134 (29%) in a non-specialised pulmonology centre. Similarities were frequent with regards to diagnostic methods including radiology and screening for infection, treatment with corticosteroids, use of high-flow oxygen and non-invasive ventilation in critical ill patients and palliative strategies. However, differences were significant in terms of the use of KL-6 and pathogen testing in urine, treatments with cyclosporine and recombinant thrombomodulin, extracorporeal membrane oxygenation in critical ill patients as well as antacid medication and anaesthesia measures as preventive methods. Conclusion: Despite the absence of recommendations, approaches to the prevention, diagnosis and treatment of AE-IPF are comparable in specialised and non-specialised ILD centres, yet certain differences in the managements of AE-IPF exist. Clinical trials and guidelines are needed to improve patient care and prognosis in AE-IPF.

Original languageEnglish
Article number699644
JournalFrontiers in Medicine
Publication statusPublished (in print/issue) - 27 Sept 2021

Bibliographical note

Copyright © 2021 Polke, Kondoh, Wijsenbeek, Cottin, Walsh, Collard, Chaudhuri, Avdeev, Behr, Calligaro, Corte, Flaherty, Funke-Chambour, Kolb, Krisam, Maher, Molina Molina, Morais, Moor, Morisset, Pereira, Quadrelli, Selman, Tzouvelekis, Valenzuela, Vancheri, Vicens-Zygmunt, Wälscher, Wuyts, Bendstrup and Kreuter.


  • idiopathic pulmonary fibrosis
  • acute exacerbation
  • questionnaire
  • pulmonologists
  • specialised ILD centres
  • non-specialised ILD centres


Dive into the research topics of 'Management of Acute Exacerbation of Idiopathic Pulmonary Fibrosis in Specialised and Non-specialised ILD Centres Around the World'. Together they form a unique fingerprint.

Cite this