TY - JOUR
TI - Acute exacerbation of idiopathic pulmonary fibrosis: International survey and call for harmonisation
AU - Kreuter, M.
AU - Polke, M.
AU - Walsh, S.L.F.
AU - Krisam, J.
AU - Collard, H.R.
AU - Chaudhuri, N.
AU - Avdeev, S.
AU - Behr, J.
AU - Calligaro, G.
AU - Corte, T.
AU - Flaherty, K.
AU - Funke-Chambour, M.
AU - Kolb, M.
AU - Kondoh, Y.
AU - Maher, T.M.
AU - Molina, M.M.
AU - Morais, A.
AU - Moor, C.C.
AU - Morisset, J.
AU - Pereira, C.
AU - Quadrelli, S.
AU - Selman, M.
AU - Tzouvelekis, A.
AU - Valenzuela, C.
AU - Vancheri, C.
AU - Vicens-Zygmunt, V.
AU - Wälscher, J.
AU - Wuyts, W.
AU - Wijsenbeek, M.
AU - Cottin, V.
AU - Bendstrup, E.
JO - European Respiratory Journal
PY - 2020
VL - 55
TODO - 4
SP - null
PB - European Respiratory Society
SN - 0903-1936, 1399-3003
TODO - 10.1183/13993003.01760-2019
TODO - brain natriuretic peptide;  cyclophosphamide;  cyclosporine;  D dimer;  methylprednisolone;  prednisolone;  rituximab;  tacrolimus, acute exacerbation of idiopathic pulmonary fibrosis;  Article;  computer assisted tomography;  controlled study;  diagnostic procedure;  disease exacerbation;  disease severity;  extracorporeal oxygenation;  fibrosing alveolitis;  health care need;  human;  immunosuppressive treatment;  invasive procedure;  major clinical study;  palliative therapy;  patient education;  priority journal;  prognosis;  pulmonary rehabilitation
TODO - 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. Copyright © ERS 2020
ER -