Περίληψη:
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
Συγγραφείς:
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, M.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.
Wijsenbeek, M.
Cottin, V.
Bendstrup, E.
Λέξεις-κλειδιά:
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
