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 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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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)

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

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