CONTEXT: Publication from the analysis of the first 500 patients enrolled in a pulmonary arterial hypertension (PAH) registry showed promising treatment results in stabilising or reducing their 1-year mortality risk | Good news for people suffering from what is a rapidly degenerative disease with no known cure to date.
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1. “SOUTH SAN FRANCISCO, Calif., April 6, 2021 /PRNewswire/ — Findings from an analysis of the first 500 patients enrolled in the SPHERE registry (SelexiPag: tHe usErs dRug rEgistry) found more than three-quarters (76%) of pulmonary arterial hypertension (PAH) patients treated with UPTRAVI® (selexipag) either maintained (56%) or reduced (20%) their one-year mortality risk score.”
2. “For individuals with PAH, risk assessment is necessary to evaluate disease progression and inform treatment decisions based on patients’ prognosis.1 UPTRAVI is an oral prostacyclin pathway agent (PPA) indicated for the treatment of patients with PAH (World Health Organization [WHO] Group I, functional class [FC] II-III) to delay disease progression and reduce the risk of hospitalization.”
3. “Additionally, achieving and/or maintaining a low-risk profile is a recommended adequate treatment response for patients with PAH.2. In the analysis of the data from the SPHERE registry, risk categories for one-year mortality are assigned to patients using two different assessments, the Comparative, Prospective Registry of Newly Initiated Therapies for Pulmonary Hypertension (COMPERA) and the Registry to Evaluate Early and Long-Term PAH Disease Management (REVEAL)† risk calculators, and PAH patients are classified as low, intermediate or high risk.”
4. “Data from this SPHERE analysis suggest that 87.8% of patients titrated UPTRAVI at a slower rate than 200-mcg BID increments, revealing that in the real-world environment patients were titrated less than the recommended titration schedule in the GRIPHON study.3,4. “We’re proud to support SPHERE, as data from this registry furthers our understanding of UPTRAVI within a clinical setting in the U.S.,” said Siân Walker‡, Head of Medical Affairs, Janssen U.S., Pulmonary Hypertension.”
5. “In the US, UPTRAVI is indicated for the treatment of PAH to delay disease progression and reduce the risk of PAH-related hospitalization.2 In Europe, UPTRAVI is indicated for the long-term treatment of PAH in adult patients with WHO FC II–III, either as combination therapy in patients insufficiently controlled with an endothelin receptor antagonist (ERA) and/or a phosphodiesterase type 5 inhibitor (PDE-5), or as monotherapy in patients who are not candidates for these therapies.”