Abstract
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.
Footnotes
This manuscript has recently been accepted for publication in the European Respiratory Journal. It is published here in its accepted form prior to copyediting and typesetting by our production team. After these production processes are complete and the authors have approved the resulting proofs, the article will move to the latest issue of the ERJ online. Please open or download the PDF to view this article.
Conflict of interest: Dr. Kreuter reports grants and personal fees from Roche, grants and personal fees from Galapagos, grants and personal fees from Boehringer, outside the submitted work.
Conflict of interest: Dr. Polke has nothing to disclose.
Conflict of interest: Dr. Walsh reports personal fees from Sanofi-Aventis, personal fees from Roche, grants and personal fees from Boehringer Ingelheim, personal fees from Galapagos, personal fees from OSIC, personal fees from Bracco, outside the submitted work.
Conflict of interest: Dr. Krisam has nothing to disclose.
Conflict of interest: Dr. Collard reports personal fees from Bayer, personal fees from Boehringer Ingelheim, personal fees from Bristol-Myers Squibb, personal fees from Global Blood Therapeutics, personal fees from ImmuneWorks, personal fees from Navitor, personal fees from Parexel, personal fees from Prometic, grants from Pulmonary Fibrosis Foundation, grants and personal fees from Three Lakes Partners, personal fees from Toray, personal fees from Unity, personal fees from Patara, personal fees from Veracyte, personal fees from Roche/Genentech, personal fees from aTyr, personal fees from Advance Medical, personal fees from MedImmune, outside the submitted work.
Conflict of interest: Dr. Chaudhuri reports grants from Boehringer Ingelheim, grants from Roche, other from Boehringer Ingelheim, other from Roche, outside the submitted work.
Conflict of interest: Dr. Avdeev has nothing to disclose.
Conflict of interest: Dr. Behr has nothing to disclose.
Conflict of interest: Dr. Calligaro has nothing to disclose.
Conflict of interest: Dr. Corte reports grants, personal fees and non-financial support from Boehringer, grants and personal fees from Roche, grants from Galapagos, personal fees from Astra Zeneca, grants from Actelion, grants from Bayer, grants from Sanofi, outside the submitted work.
Conflict of interest: Dr. Flaherty reports grants and personal fees from Boehringer Ingelheim, grants and personal fees from Roche/Genentech, personal fees from Bellerophon, personal fees from Respivant, personal fees from Veracyte, personal fees from Sanofi-Genzyme, personal fees from Blade Therapeutics, personal fees from Celgene, outside the submitted work.
Conflict of interest: Dr. Funke-Chambour has nothing to disclose.
Conflict of interest: Dr Kondoh reports advisory board fees and lecture fees from Asahi Kasei Pharma Corp., advisory board fees and lecture fees from Boehringer Ingelheim Co., Ltd., advisory board fees from Janssen Pharmaceutical K.K., lecture fees from Eisai inc., lecture fees from KYORIN Pharmaceutical Co., Ltd., lecture fees from Mitsubishi Tanabe Pharma, lecture fees from Novartis Pharma K.K., lecture fees from Shionogi & Co.,, Ltd., outside the presentation work.
Conflict of interest: TMM has, via his institution, received industry-academic funding from GlaxoSmithKline R&D and UCB and has received consultancy or speakers fees from Apellis, Astra Zeneca, Bayer, Blade Therapeutics, Boehringer Ingelheim, Bristol-Myers Squibb, Galapagos, GlaxoSmithKline R&D, Indalo, Novartis, Pliant, ProMetic, Respivnat, Roche, Samumed and UCB.
Conflict of interest: Dr. Morais has nothing to disclose.
Conflict of interest: Dr. Moor has nothing to disclose.
Conflict of interest: Dr. Morisset reports personal fees from Hoffmann La roche, personal fees from Boehringer Ingelheim, outside the submitted work.
Conflict of interest: Dr. Pereira has nothing to disclose.
Conflict of interest: Dr. Quadrelli has nothing to disclose.
Conflict of interest: Dr. Selman has nothing to disclose.
Conflict of interest: Dr. Tzouvelekis has nothing to disclose.
Conflict of interest: Dr. VALENZUELA reports personal fees from ROCHE, personal fees from BOEHRINGER INGELHEIM, personal fees from GALAPAGOS, outside the submitted work.
Conflict of interest: Dr. Vancheri reports grants and personal fees from Roche, grants and personal fees from Boehringer Ingelheim, outside the submitted work.
Conflict of interest: Dr. Vicens-Zygmunt has nothing to disclose.
Conflict of interest: Dr. Wälscher has nothing to disclose.
Conflict of interest: Dr. Wuyts has nothing to disclose.
Conflict of interest: Dr. Cottin reports personal fees and non-financial support from Actelion, grants, personal fees and non-financial support from Boehringer Ingelheim, personal fees from Bayer / MSD, personal fees from Gilead, personal fees from Novartis, grants, personal fees and non-financial support from Roche, personal fees from Sanofi, personal fees from Promedior, personal fees from Celgene, personal fees from Galapagos, personal fees from Galecto, outside the submitted work.
Conflict of interest: Dr. Bendstrup reports grants and personal fees from Boehrringer Ingelheim, grants and personal fees from Roche, outside the submitted work.
Conflict of interest: Dr. Wijsenbeek reports grants and other from Boehringer Ingelheim , grants and other from Hoffman la Roche , other from Galapagos, other from Novartis , outside the submitted work.
Conflict of interest: Dr. MOLINA-MOLINA reports grants and personal fees from Roche, Boehringer Ing, Esteve-Teijin, grants from GSK, Astra-Zeneca, personal fees from Pfizer, Chiesi, outside the submitted work.
Conflict of interest: Dr. Kolb reports grants and personal fees from Roche, grants and personal fees from Boehringer Ingelheim , grants and personal fees from GSK, grants and personal fees from Gilead, grants from Actelion, grants from Respivert, personal fees from Genoa, grants from Alkermes, grants from Pharmaxis, grants and personal fees from Prometic, personal fees from Indalo, personal fees from Third Pole, outside the submitted work.
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- Received September 6, 2019.
- Accepted January 6, 2020.
- Copyright ©ERS 2020