Acute exacerbation of idiopathic pulmonary fibrosis: international survey and call for harmonisation
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.
Source: European Respiratory Journal - Category: Respiratory Medicine Authors: Kreuter, M., Polke, M., Walsh, S. L. F., Krisam, J., Collard, H. R., Chaudhuri, N., Avdeev, S., Behr, J., Calligaro, G., Corte, T., Flaherty, K., Funke-Chambour, M., Kolb, M., Kondoh, Y., Maher, T. M., Molina Molina, M., Morais, A., Moor, C. C., Morisset, Tags: Interstitial and orphan lung disease Original Articles: Interstitial lung disease Source Type: research
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