The European Commission approved Shire’s hereditary angioedema (HAE) drug Cinryze® (C1 esterase inhibitor [human]) for use in children as young as 2 years of age. The label extension for Cinryze covers the routine prevention of angioedema attacks in children aged 6 years and above with severe and recurrent attacks of HAE who cannot tolerate or are not adequately protected by oral preventative treatments, or patients who are inadequately managed with repeated acute treatment. Shire says Cinryze is the only HAE treatment with this indication in pediatric patients. Clearance also covers use of Cinryze for the treatment and pre-procedure prevention of angioedema attacks in children with HAE who are aged 2 years and over.
Shire acquired Cinryze and the FDA- and EC-approved HAE therapy Firazyr® (icatibant injection) in 2013, through its $4.2 billion takeover of ViroPharma.
Cinryze has been approved in Europe since 2011 for use in adult and adolescent HAE patients aged 12 years and over. “This pediatric label expansion demonstrates our ongoing commitment to improving the lives of patients of all ages living with HAE,” said Philip J. Vickers, Ph.D., head of R&D at Shire. “We believe the future of HAE means preventing attacks before they happen, and we are proud to now be able to offer the first long-term preventative treatment for pediatric patients. As we expand our HAE portfolio, we remain focused on innovative solutions that fulfil unmet needs for people worldwide living with this rare disease.”
Shire reported a 30% increase in sales of Firazyr in 2016, reaching $578.5 million. Sales of Cinryze were up 10%, at $680.2 million. The firm’s kallikrein-targeting monoclonal antibody lanadelumab (SHP643, formerly DX-2930) is in Phase III development as a long-acting prophylactic treatment for HAE in adults. Shire acquired the candidate through its potentially $6.5 billion buyout of Dyax in 2015. Last month Shire reported the publication of positive data from a Phase Ib study with lanadelumab in the New England Journal of Medicine.