Background: With no approved therapies for pulmonary hypertension (PH) associated with interstitial lung disease (PH-ILD) in Europe, we surveyed clinician perceptions on PH-ILD management and unmet need to understand current real-world practices. Methods: An online clinician survey on PH-ILD management was conducted in France, Germany, Italy, Spain, and the UK. Results: Fifty-five clinicians (78% pulmonologists) each managing a median 20 PH-ILD patients (interquartile range [IQR]=10-50), participated. Upon PH suspicion, clinicians referred a median 50% (IQR=20-73%) of patients for echocardiography alone and 35% (IQR=20-78%) for echocardiography, followed by right heart catheterisation. Upon diagnosis, a median 20% (IQR=9-30%), 40% (IQR=20-50%) and 35% (IQR=20-55%) of patients fell under the pulmonary arterial pressure ranges of 21-24mmHg, 25-34mmHg and >35mmHg respectively. Fifty percent of patients received off-label treatment for their PH and, of those, off-label phosphodiesterase-5 inhibitor (PDE-5i), endothelin receptor antagonist (ERA) and prostacyclin analogues were prescribed first-line by 78%, 9% and 7% of clinicians, respectively. Upon PDE-5i non-response, 35% of clinicians proceed with an ERA, 35% with no further therapy. Fifty-five percent of clinicians used dual therapy. Yearly median inpatient admissions and emergency visits were 2.0 (IQR=1.3-2.9) and 1.5 (IQR=1.0-2.0) respectively (n=31 responses). Most clinicians (69%) highlighted lack of efficacy or evidence for current therapies as a key gap in PH-ILD management. Conclusions: This study gives insight into real-world European PH-ILD diagnosis and management. With significant use of off-label treatment, there is a large unmet need due to lack of approved therapies. Despite updated guidelines, more evidence is needed to standardise PH-ILD management.

Support statement: The present study was supported by Ferrer Pharma Ltd. Survey respondents were compensated for completion. D. Montani, J.M. Cifrián, R.P. Rojo, H. Nunes, F. Meloni, S. Ghio, J. Cannon, A. Günther and L. Howard were compensated for contributing to the survey design, completing the survey and for providing insight on the results. Wickenstones was funded by Ferrer Pharma Ltd to conduct this survey. D. Montani, J.M. Cifrián, R.P. Rojo, H. Nunes, F. Meloni, S. Ghio, J. Cannon, A. Günther and L. Howard were compensated by Ferrer Pharma for taking part in the survey and for contributing to the design of the manuscript. Acknowledgements: We would like to thank Karl Pehk and Amar Talasila (Wickenstones Ltd.) for conducting the study and writing the manuscript.