Acute exacerbation of idiopathic pulmonary fibrosis: international survey and call for harmonisation Academic Article uri icon

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abstract

  • Acute exacerbation of idiopathic pulmonary fibrosis (AE-IPF) is an often deadly complication of IPF. No focussed international guidelines for the management of AE-IPF exist. The aim of this international survey was to assess the global variability in prevention, diagnostic and treatment strategies for AE-IPF.Pulmonologists with ILD expertise were invited to participate in a survey designed by an international expert panel.509 pulmonologists from 66 countries responded. Significant geographical variability in approaches to manage AE-IPF was found. Common preventive measures included antifibrotic drugs and vaccination. Diagnostic differences were most pronounced regarding use of Krebs von den Lungen-6 and viral testing, while high-resolution computed tomography, brain natriuretic peptide and D-dimer are generally applied. High-dose steroids are widely administered (94%); the use of other immunosuppressant and treatment strategies is highly variable. Very few (4%) responders never use immunosuppression. Antifibrotic treatments are initiated during AE-IPF by 67%. Invasive ventilation or extracorporeal membrane oxygenation are mainly used as a bridge to transplantation. Most physicians educate patients comprehensively on the severity of AE-IPF (82%) and consider palliative care (64%).Approaches to the prevention, diagnosis and treatment of AE-IPF vary worldwide. Global trials and guidelines to improve the prognosis of AE-IPF are needed.

authors

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

publication date

  • April 2020