Abstract
INTRODUCTION: Patients with connective tissue disorders (CTD) and pulmonary arterial hypertension (PAH) have a poorer prognosis than those with other PAH etiologies. This study assessed the impact of CTD on healthcare outcomes among PAH patients with and without CTD comorbidities that were treated with oral selexipag. METHODS: The study utilized Optum's de-identified Clinformatics(®) Data Mart Database (2007-2021) from January 1, 2014 to June 30, 2019, and identified patients with PAH without CTD and PAH with CTD treated with oral selexipag. Patients had ≥ 12-month baseline period with no requirement for a minimum follow-up period. Patients were followed until any of the following events: discontinuation of oral selexipag, or health plan disenrollment, or death, or presence of a diagnosis claim for CTEPH, or study end date, whichever occurred first. PAH-related hospitalizations, PAH disease progression, and healthcare utilizations and costs were assessed in the follow-up period. The Cox proportional hazards model was used to evaluate the time to hospitalization and generalized linear models were used to examine healthcare costs and utilization between the two cohorts. RESULTS: In the analysis, 237 PAH without CTD, and 80 PAH patients with CTD comorbidities prescribed oral selexipag were included. The PAH without CTD comorbidities cohort was older (65 vs. 63 years old), had proportionately less females (72 vs. 83%), and higher comorbidity burden than PAH with CTD comorbidities (mean CCI index 3 vs. 2). After adjusting for potential confounders, the risk for PAH-related hospitalization (hazard ratio (HR) 1.13, p value 0.641), all-cause hospitalization (HR 1.09, p value: 0.765), and PAH disease progression (HR 1.14, p value 0.522) between the two cohorts were similar. After adjusting for baseline demographic and clinical characteristics, PAH with CTD comorbidities incurred higher total mean all-cause PAH-related medical care costs compared to PAH without CTD comorbidities. CONCLUSIONS: In this real-world study, the risk of hospitalization and PAH disease progression were similar between the two cohorts who received oral selexipag. The results from this study corroborate findings of the GRIPHON post hoc analysis of PAH-associated CTD patients and support oral selexipag use in PAH-CTD patients.