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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; Waelscher, Julia; Wuyts, Wim; Bendstrup, Elisabeth and Kreuter, Michael (27. September 2021): Management of Acute Exacerbation of Idiopathic Pulmonary Fibrosis in Specialised and Non-specialised ILD Centres Around the World. In: Frontiers in Medicine, Vol. 8, 699644 [PDF, 1MB]


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.

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