HUMANISTIC BURDEN IN PATIENTS WITH PULMONARY HYPERTENSION ASSOCIATED WITH INTERSTITIAL LUNG DISEASE (PH-ILD) USING PATIENT-REPORTED OUTCOME MEASURES (PROMS)
Author(s)
John Fastenau, PhD, MPH1, Bonny Shah, MPH, MPharm1, Louis Holdstock, PhD1, Julia Harley, BSc2, Abid Kabir, MSc2, Alastair Hinds, BSc2, James Ford, MD3.
1Insmed Incorporated, Bridgewater, NJ, USA, 2Adelphi Real World, Bollington, United Kingdom, 3The University of North Carolina at Chapel Hill, Chapel Hill, NC, USA.
1Insmed Incorporated, Bridgewater, NJ, USA, 2Adelphi Real World, Bollington, United Kingdom, 3The University of North Carolina at Chapel Hill, Chapel Hill, NC, USA.
Presentation Documents
OBJECTIVES: To assess humanistic burden using generic and disease-specific PROMs in patients with PH-ILD and differentiation across World Health Organization Functional Class (WHO-FC) groups.
METHODS: Data were analyzed from the Adelphi Real World PH-ILD Disease Specific Programme (DSP)™, a retrospective, cross-sectional survey of specialist physicians and their patients in the US and Europe between November 2024 and June 2025. Comparisons were made using ANOVA, followed by Bonferroni-adjusted pairwise comparisons.
RESULTS: Physicians reported data on 515 patients. For patients completing a questionnaire (n=234), mean (SD) age was 64.7 (10.5) years, 44.0% were female, and 94.6% were White. Mean (SD) score for the SF-12 Physical Component Summary was 40.1 (6.8); all WHO-FC groups had scores <50, indicating below average health. Overall, mean (SD) scores for EQ-5D-5L utility and EQ-5D visual analogue scale (VAS) were 0.61 (0.24) and 56.0 (17.9), respectively. Both measures worsened with deteriorating WHO-FC, with higher scores in FC I (utility: 0.74; VAS: 68.0) versus FC III/IV (utility: 0.45; VAS: 47.0; P<0.001). Mean (SD) Living with Pulmonary Fibrosis (L-PF) Impacts domain and EmPHasis-10 scores were 48.6 (17.3) and 26.2 (9.9), respectively, and worse in FC III/IV than FC I (L-PF Impacts: 55.0 vs 40.8; EmPHasis-10: 30.5 vs 19.8; P<0.001). Mean Hospital Anxiety and Depression Scale scores indicated mild levels of anxiety and depression. Depression scores worsened with deteriorating WHO-FC (FC I [5.8] and FC II [7.3] vs FC III/IV [9.8]; P<0.001).
CONCLUSIONS: Generic and disease-specific PROMs provided complementary insights into the substantial humanistic burden on patients with PH-ILD. Generic measures indicated lower general health status in these patients. Most PROM scores differentiated across WHO-FC groups, suggesting sensitivity to worsening PH-ILD severity (eg, exercise capacity) with disease-specific instruments, (EmPHasis-10, and L-PF Impacts) being particularly sensitive to worsening WHO-FC (ie, symptoms, physical function).
METHODS: Data were analyzed from the Adelphi Real World PH-ILD Disease Specific Programme (DSP)™, a retrospective, cross-sectional survey of specialist physicians and their patients in the US and Europe between November 2024 and June 2025. Comparisons were made using ANOVA, followed by Bonferroni-adjusted pairwise comparisons.
RESULTS: Physicians reported data on 515 patients. For patients completing a questionnaire (n=234), mean (SD) age was 64.7 (10.5) years, 44.0% were female, and 94.6% were White. Mean (SD) score for the SF-12 Physical Component Summary was 40.1 (6.8); all WHO-FC groups had scores <50, indicating below average health. Overall, mean (SD) scores for EQ-5D-5L utility and EQ-5D visual analogue scale (VAS) were 0.61 (0.24) and 56.0 (17.9), respectively. Both measures worsened with deteriorating WHO-FC, with higher scores in FC I (utility: 0.74; VAS: 68.0) versus FC III/IV (utility: 0.45; VAS: 47.0; P<0.001). Mean (SD) Living with Pulmonary Fibrosis (L-PF) Impacts domain and EmPHasis-10 scores were 48.6 (17.3) and 26.2 (9.9), respectively, and worse in FC III/IV than FC I (L-PF Impacts: 55.0 vs 40.8; EmPHasis-10: 30.5 vs 19.8; P<0.001). Mean Hospital Anxiety and Depression Scale scores indicated mild levels of anxiety and depression. Depression scores worsened with deteriorating WHO-FC (FC I [5.8] and FC II [7.3] vs FC III/IV [9.8]; P<0.001).
CONCLUSIONS: Generic and disease-specific PROMs provided complementary insights into the substantial humanistic burden on patients with PH-ILD. Generic measures indicated lower general health status in these patients. Most PROM scores differentiated across WHO-FC groups, suggesting sensitivity to worsening PH-ILD severity (eg, exercise capacity) with disease-specific instruments, (EmPHasis-10, and L-PF Impacts) being particularly sensitive to worsening WHO-FC (ie, symptoms, physical function).
Conference/Value in Health Info
2026-05, ISPOR 2026, Philadelphia, PA, USA
Value in Health, Volume 29, Issue S6
Code
CO114
Topic
Clinical Outcomes
Disease
SDC: Respiratory-Related Disorders (Allergy, Asthma, Smoking, Other Respiratory)