WASHINGTON DC – Cinryze (C1 inhibitor-nf) replacement therapy should be considered a routine component of the management of pregnant women with hereditary angioedema (HAE), researchers said here at the 2009 Annual American Academy of Allergy, Asthma and Immunology (AAAAI).
James W. Baker, MD, with the Allergy, Asthma and Dermatology Research Center in Lake Oswego, Oregon, and colleagues made the recommendation following their research showing that the therapy was effective and safe in a small series of pregnant women with HAE.
“While HAE attacks are often spontaneous, they can be triggered by emotional or physical stress, causing attacks to increase occasionally in frequency and severity during pregnancy,” Dr. Baker said. “Unfortunately, treatment options are limited for these patients.”
“This study provides encouraging evidence for the safe and effective treatment of HAE with Cinryze during pregnancy and delivery,” he added, “and we look forward to further evaluating the use of Cinryze in pregnant women to fully understand its potential in addressing this unmet medical need.”
The currently available therapies for HAE in the U.S. are contraindicated during pregnancy or should be used with caution. While severe angioedema attacks in pregnant women may be prevented with C1 inhibitor therapy, the data to have been drawn mostly from European trials.
Dr. Baker reported data in six patients who received 1000U of Cinryze replacement therapy one to two times per week as prophylaxis of HAE attacks during pregnancy. An additional patient received Cinryze immediately before delivery and again two days later.
Results showed that all six of the women treated through pregnancy had normal healthy deliveries. The seventh woman, who received Cinryze before and after delivery, also had normal healthy delivery.
Monthly HAE episodes decreased from 29.5 to 3.9. Overall, treatment was associated with an 87% reduction in HAE episodes.
Based on the findings, Dr. Baker strongly recommended that Cinryze therapy be included in the standard care of women with HAE during their pregnancy and delivery.
He added that self-administration of Cinryze may prove to be a convenient and cost-effective mode of treatment.
Hereditary angioedema is a rare disorder caused by C1 inhibitor deficiency with an estimated prevalence between 1 in 10,000 and 1 in 50,000. The disease is characterized by episodes of subcutaneous and sub-mucosal edema.
Written by Jill Stein
Jill Stein is a Paris-based freelance medical writer.
jillstein03@gmail.com