TY - JOUR
T1 - Management of Acute Exacerbation of Idiopathic Pulmonary Fibrosis in Specialised and Non-specialised ILD Centres Around the World
AU - Polke, Markus
AU - Kondoh, Yasuhiro
AU - Wijsenbeek, Marlies
AU - Cottin, Vincent
AU - Walsh, Simon L F
AU - Collard, Harold R
AU - Chaudhuri, Nazia
AU - Avdeev, Sergey
AU - Behr, Jürgen
AU - Calligaro, Gregory
AU - Corte, Tamera J
AU - Flaherty, Kevin
AU - Funke-Chambour, Manuela
AU - Kolb, Martin
AU - Krisam, Johannes
AU - Maher, Toby M
AU - Molina Molina, Maria
AU - Morais, Antonio
AU - Moor, Catharina C
AU - Morisset, Julie
AU - Pereira, Carlos
AU - Quadrelli, Silvia
AU - Selman, Moises
AU - Tzouvelekis, Argyrios
AU - Valenzuela, Claudia
AU - Vancheri, Carlo
AU - Vicens-Zygmunt, Vanesa
AU - Wälscher, Julia
AU - Wuyts, Wim
AU - Bendstrup, Elisabeth
AU - Kreuter, Michael
N1 - 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.
PY - 2021/9/27
Y1 - 2021/9/27
N2 - 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.
AB - 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.
KW - idiopathic pulmonary fibrosis
KW - acute exacerbation
KW - questionnaire
KW - pulmonologists
KW - specialised ILD centres
KW - non-specialised ILD centres
U2 - 10.3389/fmed.2021.699644
DO - 10.3389/fmed.2021.699644
M3 - Article
C2 - 34646836
SN - 2296-858X
VL - 8
JO - Frontiers in Medicine
JF - Frontiers in Medicine
M1 - 699644
ER -