PH-ILD management and unmet need in Europe: a clinician survey

Luke Howard, Stefano Ghio, Federica Meloni, John Cannon, Hilario Nunes, Jose Cifrian-Martinez, Raquel Rojo, Andreas Günther, David Montani, Karl Pehk, Miriam Delgado


There are no approved or effective therapies for pulmonary hypertension (PH) associated with interstitial lung disease (PH-ILD) in Europe and real-world management data is lacking.

A retrospective online survey was conducted to elicit clinician insights on current disease management of PH-ILD in France, Germany, Italy, Spain, and the UK. The analysis of management pathway data is reported here.

55 clinicians (78% pulmonologist, 16% cardiologist and 5% rheumatologist) each managing 38 (± 37) PH-ILD patients, participated. When PH was suspected, 48% (± 31) of patients were referred to echocardiography (TTE) alone and 46% (± 31) were referred for TTE followed by right heart catheterisation. Upon PH diagnosis, 24% (± 22) of patients had 21–24mmHg, 36% (±19) 25–34mmHg and 40% (±24) >35mmHg. Off licence phosphodiesterase-5 inhibitor (PDE5i), endothelin receptor antagonist (ERA) and prostacyclin analogue were prescribed 1st line by 78%, 9% and 7% of clinicians respectively. Upon PDE5i failure, 36% of clinicians followed up with an ERA, 36% with no further therapy. 55% and 40% of clinicians use dual (75% being PDE5i + ERA) and triple therapy respectively. 49% (±25), 38% (±20), 32% (±23) and 11% (±10) of PH-ILD patients received anti-fibrotic, anti-inflammatory, immunosuppressant, and rituximab therapy, respectively. Most clinicians (69%) highlighted a lack of efficacy/evidence for current therapies as a key gap in PH-ILD management.

Our study has gained insight into how PH-ILD patients are diagnosed and managed in a real-world setting in Europe. There is variation in diagnosis and management, with significant use of off-label treatment in PH-ILD, indicating a large unmet need due to lack of approved therapies.