Extended sotatercept as an add-on treatment for pulmonary arterial hypertension maintained long-term benefit, with low treatment-related discontinuation.

Extended sotatercept as an add-on treatment for pulmonary arterial hypertension (PAH) showed sustained maintenance of benefit in a long-term open-label extension. The safety assessment indicated low treatment-related discontinuation.

“The PULSAR phase 2 and STELLAR phase 3 trials showed the efficacy of sotatercept in the treatment of PAH by improving hemodynamic and clinical parameters,” said Ioanna Preston, MD, at the 2023 ERS International Congress. She presented interim results of the first 409 patients included in SOTERIA, an open-label follow-up to evaluate long-term results for safety, tolerability, and efficacy. Enrollment was possible for patients taking sotatercept for PAH within various prior phase 2 and 3 studies.

At baseline, a median age of 49 and 81.9% of women were observed in the study cohort. The median time since PAH diagnosis was 8.1 years, and over 90% of the participants were already on double or triple therapy. The median extent of drug exposure was 189 days in SOTERIA, but 462 days when including treatment within the parent trial.

“Very few treatment-emergent adverse events led to treatment discontinuation,” Dr. Preston said. Telangiectasia was observed in 22.7%, and it entailed a dose-hold or reduction in four patients. However, none of the telangiectasia cases were serious or led to discontinuation of treatment. “There were 12.2% epistaxis events, but only 1% was related to telangiectasia in the opinion of the investigator,” Dr. Preston added. She also reported thrombocytopenia in 3.7%. Within the analyzed cohort, four deaths occurred. Moreover, nine events of clinical worsening that included three PAH-related hospitalizations, happened in seven patients.

As for clinical efficacy measures like change from baseline in 6-minute walking test, NT-proBNP, WHO functional class and percentage of achievement of a low French Risk Score, Dr. Preston stressed at 1-year the improvements prior achieved at 6 months were largely maintained. Also, the long-term treatment involved changes in the background therapy of the participants. Among 154 patients receiving IV prostacyclin, 22 were decreased and nine increased in dosage (≥10%). “Of note, out of 272 patients on any prostacyclin, eight patients have discontinued prostacyclin during SOTERIA,” Dr. Preston emphasized.

“The results of SOTERIA support the long-term durable benefit and safety of sotatercept for the treatment of PAH,” she concluded.

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