Management of acute exacerbation of idiopathic pulmonary fibrosis in specialised and non-specialised ILD centres around the world
Author(s)
Type
Journal Article
Abstract
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.
Date Issued
2021-09-27
Date Acceptance
2021-08-26
Citation
Frontiers of Medicine, 2021, 8
ISSN
1673-7342
Publisher
Springer Verlag
Journal / Book Title
Frontiers of Medicine
Volume
8
Copyright Statement
© 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. This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.
License URL
Sponsor
National Institute for Health Research
British Lung Foundation
Identifier
https://www.ncbi.nlm.nih.gov/pubmed/34646836
Grant Number
CS-2013-13-017
C17-3
Subjects
acute exacerbation
idiopathic pulmonary fibrosis
non-specialised ILD centres
pulmonologists
questionnaire
specialised ILD centres
Publication Status
Published
Coverage Spatial
Switzerland
Article Number
ARTN 699644