Incremental Burden of Pulmonary Hypertension Among Patients With Interstitial Lung Disease in the Real-World Setting

6 November 2025

Charles D. BurgerJasmanda WuGerald O'BrienNatalya MakulovaTrevor WardMeghana G. ShamsunderBonny ShahZeenat Safdar

https://doi.org/10.1002/pul2.70201

Abstract
Pulmonary hypertension (PH) is a common complication in interstitial lung disease (ILD), but the additional burden it imposes on patients and healthcare systems is not well characterized. This retrospective analysis of claims data from the US Merative MarketScan database assessed hospitalization rates and costs over 2 years in patients with connective tissue disease-related ILD (CTD-ILD) and non-CTD-ILD with or without PH (between January 2017 and December 2019). Index was the date of first ILD claim; baseline was the 12-month preindex period. In total, 16,129 patients with non-CTD-ILD (1502 [9%] with PH) and 4545 patients with CTD-ILD (663 [15%] with PH) were identified. A higher proportion of patients with non-CTD-ILD with PH, compared with patients without PH, had all-cause and heart failure (HF)-related hospitalizations during baseline (all-cause, 37.0% vs. 22.3%; HF-related, 14.2% vs. 3.1%), 0–12 months (62.4% vs. 43.0%; 29.0% vs. 7.0%), and 13–24 months (44.7% vs. 18.3%; 21.5% vs. 3.2%) follow-up (p < 0.0001 for all). A significantly higher proportion of patients with CTD-ILD with PH, compared with patients without PH, had all-cause and HF-related hospitalizations during baseline (all-cause: 30.0% vs. 20.6%; HF-related: 9.1% vs. 1.9%), 0–12 months (41.9% vs. 29.1%; 15.5% vs. 3.9%), and 13–24 months (37.9% vs. 18.9%; 13.9% vs. 2.5%) follow-up (p < 0.0001 for all). In both cohorts, total all-cause and HF-related costs were significantly higher in patients with PH at baseline, 0–12 months, and 13–24 months follow-up (p < 0.05 for all). PH substantially increased hospitalization risks and costs in both types of ILD, underscoring the importance of improving outcomes in patients with ILD-PH. 

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