Cinryze™ Replacement Therapy in Hereditary Angioedema and Pregnancy

RATIONALE: Pregnancy may increase the number and severity of hereditary angioedema (HAE) attacks. Current therapies available in the United States are mainly contraindicated or should be used with caution. Severe angioedema attacks in pregnant women may be prevented with C1 inhibitor replacement therapy. METHODS: Six patients received 1000U of Cinryze (C1 inhibitor-nf) replacement therapy 1-2 times per week as prophylaxis of HAE attacks during pregnancy. An additional patient received Cinryze 1000U immediately prior to delivery and again two days later. Data on frequency of HAE attacks and number of emergency medical visits prior to and following initiation of Cinryze replacement therapy were gathered. Side effects were monitored . RESULTS: Six of the pregnant women had normal healthy deliveries and the seventh, currently in her second trimester, remains on Cinryze. No patient treated with Cinryze replacement therapy suffered HAE related complications during her pregnancy. Number of attacks and number of emergency medical visits were reduced by >85%. No adverse events secondary to Cinryze were reported. CONCLUSIONS: Cinryze replacement therapy is a safe and effective treatment of HAE in pregnancy and should be considered as part of the standard of care during pregnancy and delivery for patients with HAE.