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 Molina; Morais, Antonio; Moor, Catharina C; Morisset, Julie; Pereira, Carlos; ... (2020). Acute exacerbation of idiopathic pulmonary fibrosis: International survey and call for harmonisation. European respiratory journal, 55(4) European Respiratory Society 10.1183/13993003.01760-2019
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Funke-Chambour M_Acute exacerbation of idiopathic.pdf - Accepted Version Available under License Publisher holds Copyright. Download (8MB) | Preview |
AIM
Acute exacerbation of idiopathic pulmonary fibrosis (AE-IPF) is an often deadly complication of IPF. No focused 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.
MATERIAL AND METHODS
Pulmonologists with ILD expertise were invited to participate in a survey designed by an international expert panel.
RESULTS
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 KL-6 and viral testing, while HRCT, BNP 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%).
CONCLUSION
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.
Item Type: |
Journal Article (Original Article) |
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Division/Institute: |
04 Faculty of Medicine > Department of Gastro-intestinal, Liver and Lung Disorders (DMLL) > Clinic of Pneumology |
UniBE Contributor: |
Funke-Chambour, Manuela |
Subjects: |
600 Technology > 610 Medicine & health |
ISSN: |
0903-1936 |
Publisher: |
European Respiratory Society |
Language: |
English |
Submitter: |
Heidi Lobsiger |
Date Deposited: |
09 Mar 2020 16:01 |
Last Modified: |
05 Dec 2022 15:37 |
Publisher DOI: |
10.1183/13993003.01760-2019 |
PubMed ID: |
32060068 |
BORIS DOI: |
10.7892/boris.140888 |
URI: |
https://boris.unibe.ch/id/eprint/140888 |