The U.S. Food and Drug Administration (FDA) has approved an update to the product label of Winrevair (sotatercept) for the treatment of adults with pulmonary arterial hypertension (PAH).
PAH is a rare lung and heart condition characterized by abnormally high blood pressure in the pulmonary artery. Symptoms include shortness of breath during exercise and fainting spells. The symptoms tend to get worse over time and may include dizziness, swelling of the ankles or legs, chest pain, and a racing pulse. Some cases of PAH are due to genetic changes in the BMPR2 gene. PAH can also occur secondary to underlying conditions such as connective tissue diseases, HIV infection, chronic hemolytic anemia, and congenital heart disease. PAH can also be induced by certain drugs and toxins.
Sotatercept is an activin signaling inhibitor therapy originally approved for the treatment of PAH to improve exercise capacity in March 2024. The expanded indication now includes sotatercept treatment to reduce risk of clinical worsening events such as hospitalization, lung transplantation, or death. The approval update follows results from the ZENITH clinical trial.
The ZENITH clinical trial is a phase 3 global, double-blind, placebo-controlled, multicenter, parallel-group study of 172 adult patients with PAH (WHO functional class III or IV) who have a high risk of mortality. The addition of sotatercept to background therapy demonstrated a statistically significant and clinically meaningful reduction of 76% in the risk of major morbidity and mortality outcomes compared to placebo.
Additionally, time to first occurrence of all-cause death, lung transplantation, or PAH-worsening hospitalization of greater than or equal to 24 hours, occurred in 15 patients treated with sotatercept. This is compared to occurrence in 47 patients treated with placebo.
The most common adverse reactions in the ZENITH study were infections, epistaxis, diarrhea, telangiectasia, increased hemoglobin, rash, erythema, and gingival bleeding. The median duration of exposure was 435 days in the sotatercept group and 268 days in the placebo group. Compared to the four patients who discontinued treatment in the placebo group due to adverse events, only one patient discontinued in the sotatercept group.
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To learn more about PAH and other rare lung conditions, visit https://checkrare.com/diseases/lung-diseases/
