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*

*Corresponding author for this work

Research output: Contribution to journalArticleAcademicpeer-review

8 Citations (Scopus)
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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 - 27 Sept 2021

Bibliographical note

Funding Information:
We would like to thank all participants who generously agreed to participate in this study.

Publisher Copyright:
© 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.


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