Shire bags paediatric label expansion for Cinryze in the US

US regulators have approved a label expansion for Shire’s Cinryze, allowing its use in children aged six and above with hereditary angioedema.

HEA is a rare, debilitating and potentially life-threatening rare genetic disease that causes swelling in the face, extremities and GI tract, thought to affect around

Cinryze (C1 inhibitor [human]), acquired by Shire through its purchase of ViroPharma in 2014, works by raising plasma levels of C1-INH in patients with the condition, who are prone to swelling due to an underlying deficiency of this protein.

The therapy has been on the market since 2008 for preventing HAE attacks in older patients with the condition; its expansion to include younger patients was based on the Phase III study 0624-301, which met its primary endpoint of monthly-normalised number of attacks.

Overall the safety and tolerability of Cinryze has been shown to be similar in clinical studies of paediatric, adolescent and adult patients with HAE, the firm noted.

“Symptoms of HAE often present in childhood with the average child experiencing their first HAE attack around the age of 10. With the FDA label expansion of Cinryze, children as young as 6 years old living with HAE now have the first FDA approved treatment option available to help prevent attacks,” said Andreas Busch, head of R&D at Shire.