RT Journal Article
SR Electronic
T1 Acute exacerbation of idiopathic pulmonary fibrosis: international survey and call for harmonisation
JF European Respiratory Journal
JO Eur Respir J
FD European Respiratory Society
SP 1901760
DO 10.1183/13993003.01760-2019
VO 55
IS 4
A1 Michael Kreuter
A1 Markus Polke
A1 Simon L.F. Walsh
A1 Johannes Krisam
A1 Harold R. Collard
A1 Nazia Chaudhuri
A1 Sergey Avdeev
A1 Jürgen Behr
A1 Gregory Calligaro
A1 Tamera Corte
A1 Kevin Flaherty
A1 Manuela Funke-Chambour
A1 Martin Kolb
A1 Yasuhiro Kondoh
A1 Toby M. Maher
A1 Maria Molina Molina
A1 Antonio Morais
A1 Catharina C. Moor
A1 Julie Morisset
A1 Carlos Pereira
A1 Silvia Quadrelli
A1 Moises Selman
A1 Argyrios Tzouvelekis
A1 Claudia Valenzuela
A1 Carlo Vancheri
A1 Vanesa Vicens-Zygmunt
A1 Julia Wälscher
A1 Wim Wuyts
A1 Marlies Wijsenbeek
A1 Vincent Cottin
A1 Elisabeth Bendstrup
YR 2020
UL http://erj.ersjournals.com/content/55/4/1901760.abstract
AB 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.Lack of focussed international guidelines for management of acute exacerbation of IPF results in global variability in prevention, diagnosis and treatment strategies. Global trials are urgently needed to inform international specific guidelines for AE-IPF. http://bit.ly/3a8FB5i