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

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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.

authors

  • Polke, Markus
  • Kondoh, Yasuhiro
  • Wijsenbeek, Marlies
  • Cottin, Vincent
  • Walsh, Simon LF
  • Collard, Harold R
  • Chaudhuri, Nazia
  • Avdeev, Sergey
  • Behr, Jürgen
  • Calligaro, Gregory
  • Corte, Tamera J
  • Flaherty, Kevin
  • Funke-Chambour, Manuela
  • Kolb, Martin Rainer
  • 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

publication date

  • January 2021