24/7 Market News- Scholar Rock Reports Positive Phase 3 SAPPHIRE Study Results in Patients with SMA

DENVER, Colo., Oct 07, 2024 (247marketnews.com)- Scholar Rock (NASDAQ:SRRK) reported that its Phase 3 SAPPHIRE clinical trial (NCT05156320) evaluating the efficacy and safety of apitegromab, an investigational muscle-targeted therapy, in patients with SMA, achieved its primary endpoint by demonstrating a statistically significant and clinically meaningful improvement for apitegromab versus placebo in motor function as measured by the gold standard HFMSE in SMA patients on chronic dosing of standard of care therapies (either nusinersen or risdiplam).

Jay Backstrom, M.D., MPH, Scholar Rock’s President and CEO, commented, “We are thrilled that apitegromab met the primary endpoint in our Phase 3 SAPPHIRE clinical study. The results clearly demonstrate robust and clinically meaningful improvement in motor function in patients with SMA.

“At Scholar Rock, we are working with urgency to deliver the potentially transformative benefits of apitegromab to children and adults with SMA in the US, Europe, and around the world.”

Thirty percent of patients receiving apitegromab had >3 point improvement in HFMSE compared to 12.5% of placebo group’s patients.

Jing Marantz, M.D., Ph.D., Scholar Rock’s Chief Medical Officer, added, “We are grateful to the families and investigators who participated in our trials. The positive Phase 3 SAPPHIRE trial, along with over 4 years of TOPAZ clinical trial data, clearly demonstrate the potentially transformative benefit of apitegromab to drive clinically meaningful improvements in motor function as measured by HFMSE in a broad SMA population, where motor function would normally be expected to generally decline over time.”

The U.S. Food and Drug Administration (FDA) granted apitegromab Fast Track, Orphan Drug, and Rare Pediatric Disease designations, for the treatment of SMA, while the European Medicines Agency (EMA) granted Priority Medicines (PRIME) and Orphan Medicinal Product designations, to apitegromab for the treatment of SMA.

Scholar Rock plans to submit a U.S. Biologics License Application (BLA) and a European Union marketing authorisation application (MAA) in Q1 2025.

Kenneth Hobby, President of Cure SMA, exclaimed, “It’s a great day for people living with SMA and their families. These encouraging trial results mark a critical milestone for the SMA community.

“Declining motor function and hopes for reversing losses associated with muscle weakness are significant unmet needs, impacting activities of daily living, from breathing, eating, self-care, to working and social interactions. We need an approved therapy that can support motor function and further improve daily activities for people with SMA.”

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