Shire Receives European Approval for Label Extension of CINRYZE to Prevent and Treat Hereditary Angi

Shire announced that the European Commission (EC) has approved a label extension granting three new indications for CINRYZE^® (C1 inhibitor [human]), broadening its use to children with Hereditary angiodema (HAE), a rare, genetic disorder that results in recurring attacks of edema (swelling). The body sites most commonly affected are mainly the extremities and abdomen. CINRYZE is now indicated for routine prevention of angioedema attacks in children (ages 6 years and above) with severe and recurrent attacks of HAE who are intolerant to or insufficiently protected by oral preventions treatments, or patients who are inadequately managed with repeated acute treatment. It is the first and only HAE treatment with this indication in pediatric patients. CINRYZE is also now approved for the treatment and pre-procedure prevention of angioedema attacks in children (ages 2 years and above) with HAE.

Symptoms of HAE often present in childhood, and while attacks can occur at any age, early onset may predict a more severe disease course. Attacks often occur in children without a clear trigger,and may affect a child's participation in school, activities, and sports, which can leave them feeling socially isolated. Less frequently, HAE can cause life-threatening attacks due to obstruction in the upper airways.

"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, Shire. "We believe the future of HAE means preventing attacks before they happen, and 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."

CINRYZE has been approved since 2011 for these indications in adults and adolescents ages 12-17 years with HAE.

Henrik Balle Boysen, Executive Director of HAEi stated, "Over the years we have encountered many children who suffer from frequent and severe HAE symptoms that often occur spontaneously and without warning. Despite improvements in the management of HAE in recent years, this new long-term prophylaxis indication for alleviating the frequency of HAE symptoms will be a welcome addition for families with HAE in Europe."

CINRYZE will be available for use in pediatric patients later in 2017 throughout Member States of the European Union (EU), as well the European Economic Area (EEA) in which Shire currently has a license in the adult and adolescent population.