Unmet need in pulmonary hypertension-associated interstitial lung disease (PH-ILD): a clinician survey of real-world management of PH-ILD in Europe

Abstract

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: 55 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 21-24 mmHg, 25-34 mmHg and >35 mmHg, respectively. 50% 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. 55% 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.

FIGURE 1

Diagnostic procedures carried out by clinicians to confirm PH in ILD patients suspected to have PH (n=55). EU5: European five countries (France, Germany, Italy, Spain and the UK); ILD: interstitial lung disease; PH: pulmonary hypertension; RHC: right heart catheterisation.

FIGURE 2

Distribution of a) clinician prescription of ILD drugs in PH-ILD patients and b) clinician-reported PH-ILD patient receipt of ILD therapies (n=16–55^#). “Others” consists of cyclophosphamide and immunoglobulins. ILD: interstitial lung disease; PH-ILD: pulmonary hypertension associated with interstitial lung disease; TNF: tumour necrosis factor. ^#: the four clinicians that stated that ILD treatment was not known were not followed with the questions in b; only those that stated that a given ILD drug was being used in their patients in a were given the follow-up question for the specific drug in b.

FIGURE 3

Off-label PAH treatment frequency in PH-ILD patients managed by clinicians (n=53^#). Each circle represents one clinician's answer; bar represents the mean value. PAH: group 1 pulmonary hypertension; PH-ILD: pulmonary hypertension associated with interstitial lung disease; EU5: European five countries (France, Germany, Italy, Spain and the UK). ^#: two clinicians did not answer this question.

FIGURE 4

Off-label PAH monotherapy frequency in PH-ILD patients managed by clinicians (n=55). Each circle represents one clinician's answer; bar represents the mean value. PDE-5: phosphodiesterase-5; PAH: group 1 pulmonary hypertension; PH-ILD: pulmonary hypertension associated with interstitial lung disease; sGC: soluble guanyl cyclase.

FIGURE 5

Clinician-selected prognostic factors at diagnosis of pulmonary hypertension (PH) for a positive response to off-label PAH therapy in PH-ILD patients (n=55). ILD: interstitial lung disease; PAH: group 1 pulmonary hypertension.

FIGURE 6

Median clinician-reported lung transplantation rates for their PH-ILD patients (n=55). PH-CTD-ILD: pulmonary hypertension associated with connective tissue disease-based interstitial lung disease; PH-ILD: pulmonary hypertension associated with interstitial lung disease; PH-IPF: pulmonary hypertension associated with idiopathic pulmonary fibrosis.