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.
- Kreuter, M.
- Polke, M.
- Walsh, S. L. F.
- Krisam, J.
- Collard, H. R.
- Chaudhuri, N.
- Avdeev, S.
- Behr, J.
- Calligaro, G.
- Corte, T.
- Flaherty, K.
- Funke-Chambour, M.
- Kolb, M.
- Kondoh, Y.
- 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.
- Walscher, J.
- Wuyts, W.
- Wijsenbeek, M.
- Cottin, V.
- Bendstrup, E.
