OBJECTIVES: Pulmonary Hypertension associated with Lung Disease and/or Hypoxia (PH-LD), including PH with interstitial lung disease (PH-ILD), are severe conditions with a high mortality. However, data on epidemiology and healthcare management in the real-world setting are limited.To estimate the epidemiology and the healthcare resource use (HCRU) of patients with PH-LD/ILD.

METHODS: We conducted a retrospective observational study using the UK Clinical Practice Research Datalink (CPRD) and Hospital Episode Statistics (HES), including >18 million patients. An algorithm, developed with six PH medical experts, identified PH-LD and PH-ILD patients from January 2017 to December 2019 based on a combination of PH and LD ICD-10 codes. The index date was the first PH diagnosis, with a 24-month minimum lookback period and up to 60 months follow-up until end of study, loss to follow-up or death.

RESULTS: A total of 6,690 patients with PH-LD (mean age 71, 54% female) and 1,561 with PH-ILD (mean age 71, 62% female) were included. The estimated period prevalence (2017-2019) and annual incidence of PH-LD was 2.65 (95%CI 2.59-2.70) and 0.67 (0.651-0.68) per 10,000 people, and PH-ILD 0.66 (95%CI 0.64-0.69) and 0.16 (0.15-0.16), respectively. During the first-year of follow-up, incident PH-LD and PH-ILD patients had a mean (95%CI) 3.46 (3.40-3.51) and 2.93 (2.82-3.03) all-cause inpatient hospitalizations per person-year, including 1.87 (1.83-1.91) and 1.78 (1.70-1.87) due to PH, and 2.94 (2.89-2.99) and 2.51 (2.41-2.60) due to LD. Mean length of stay (SD) was 10 (15) and 10 (13) days, respectively. Hospitalization rates decreased over time. Median overall survival (95%CI) for PH-LD and PH-ILD patients was 19.3 months (17.77-20.8) and 15.1 months (12.66-18.2), respectively.

CONCLUSIONS: With current therapies patients with PH-LD and PH-ILD have a significant unmet need with high levels of HCRU and a poor prognosis.