Acute exacerbation of idiopathic pulmonary fibrosis: international survey and call for harmonisation

Kreuter, Michael; Polke, Markus; Walsh, Simon L. F.; Krisam, Johannes; Collard, Harold R.; Chaudhuri, Nazia; Avdeev, Sergey; Behr, Jürgen; Calligaro, Gregory; Corte, Tamera; Flaherty, Kevin; Funke-Chambour, Manuela; Kolb, Martin; Kondoh, Yasuhiro; Maher, Toby M.; Molina, Maria; Morais, Antonio; Moor, Catharina C.; Morisset, Julie; Pereira, Carlos; Quadrelli, Silvia; Selman, Moises; Tzouvelekis, Argyrios; Valenzuela, Claudia; Vancheri, Carlo; Vicens-Zygmunt, Vanesa; Waelscher, Julia; Wuyts, Wim; Wijsenbeek, Marlies; Cottin, Vincent und Bendstrup, Elisabeth (2020): Acute exacerbation of idiopathic pulmonary fibrosis: international survey and call for harmonisation. In: European Respiratory Journal, Bd. 55, Nr. 4, 1901760

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.

• Dokumententyp: Zeitschriftenartikel
• Fakultät: Medizin
• Themengebiete: 600 Technik, Medizin, angewandte Wissenschaften > 610 Medizin und Gesundheit
• ISSN: 0903-1936
• Sprache: Englisch