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

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.

• Polke, M.
• Kondoh, Y.
• Wijsenbeek, M.
• Cottin, V.
• Walsh, S. L. F.
• Collard, H. R.
• Chaudhuri, N.
• Avdeev, S.
• Behr, J.
• Calligaro, G.
• Corte, T. J.
• Flaherty, K.
• Funke-Chambour, M.
• Kolb, M.
• Krisam, J.
• Maher, T. M.
• Molina Molina, M.
• Morais, A.
• Moor, C. C.
• Morisset, J.
• Pereira, C.
• Quadrelli, S.
• Selman, M.
• Tzouvelekis, A.
• Valenzuela, C.
• Vancheri, C.
• Vicens-Zygmunt, V.
• Wälscher, J.
• Wuyts, W.
• Bendstrup, E.
• Kreuter, M.