Characteristics of Patients with Pulmonary Arterial Hypertension Receiving Selexipag in the SPHERE Registry by Race and Ethnicity

Introduction Racial/ethnic minority populations in the US have a high burden of pulmonary arterial hypertension (PAH). Objective To evaluate demographics, disease characteristics, prescribing patterns, hospitalization, and survival in racial/ethnic groups in the SPHERE registry. Methods SPHERE was a US, multicenter, prospective, observational registry of adults prescribed selexipag in clinical practice (November 2016-March 2020). Follow-up was ≤ 18 months, with data collected at routine quarterly visits. Results There were 759 patients with PAH: 549 (72.3%) non-Hispanic White; 117 (15.4%) Black/African American; 45 (5.9%) Hispanic; and 48 (6.3%) other/unknown race/ethnicity. Overall, 50.6% of participants had idiopathic and 27.0% had connective tissue disorder-associated PAH. Median age at diagnosis in non-Hispanic White, Black/African American, and Hispanic groups was 57, 56, and 43 years, and at selexipag initiation was 62, 60, and 51 years, respectively. Hispanic participants had less severe symptoms at enrollment: 40.0% had World Health Organization functional class III versus 53.4% and 50.4% of non-Hispanic White and Black/African American participants, respectively. Comorbidities were high, with some differences between groups. No notable differences existed between groups in selexipag dose or PAH-specific therapy; overall, 30.8% received monotherapy, 55.6% dual therapy, and 8.4% triple therapy before selexipag initiation. Discontinuations resulting from selexipag-related adverse events in any group were few (2.2%-7.7%). All-cause hospitalization and survival were similar across groups. Conclusion Selexipag was prescribed as part of combination therapy for PAH regardless of differing demographics and clinical characteristics across racial/ethnic groups, with no differences in selexipag discontinuation, hospitalization, and survival

Adverse effect · Ethnic group · Observational study · Interstitial Lung Diseases and Idiopathic Pulmonary Fibrosis · Medicine · Pulmonary Hypertension Research and Treatments · Epidemiology · Internal Medicine