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

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

JOURNAL=Frontiers in Medicine

VOLUME=8

YEAR=2021

URL=https://www.frontiersin.org/journals/medicine/articles/10.3389/fmed.2021.699644

DOI=10.3389/fmed.2021.699644

ISSN=2296-858X

ABSTRACT=<p><bold>Background:</bold> 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.</p><p><bold>Material and Methods:</bold> 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.</p><p><bold>Results:</bold> 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.</p><p><bold>Conclusion:</bold> 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.</p>