Berinert treatment prevents HAE swelling attacks during childbirth
Study backs use of short-term therapy, even amid expanding options
Short-term preventive treatment with on-demand Berinert (human C1 esterase inhibitor) immediately before delivery helps women with hereditary angioedema (HAE) avoid swelling attacks during childbirth, according to case series and literature review from Japan.
About half of pregnant women with HAE experienced more acute attacks during pregnancy, particularly in the early and late trimesters, all of which were successfully managed with short-term preventive treatment with Berinert.
“[Berinert] was given [one hour] before delivery, with several additional doses prepared,” the researchers wrote. “This strategy successfully prevented peripartum attacks and ensured safe outcomes.”
Case and review details were published in the Journal of Dermatology, in a study, titled “Perinatal Management and Clinical Outcomes of Hereditary Angioedema in Japan: A Case Series and Comprehensive Literature Review.”
Pregnancy impacts HAE differently in each woman
HAE is a genetic disease marked by recurrent episodes of swelling in the deeper layers of the skin or the mucus membranes. It most commonly affects the face, abdomen, hands, feet, and genitals.
Pregnancy impacts HAE differently in each woman, with some experiencing an increase in the frequency and severity of attacks, while others see an improvement or no change.
Despite advances in medications and the monitoring of HAE during the perinatal period — pregnancy, childbirth, and postpartum — standardized management protocols in Japan are lacking.
To address this gap, a research team in Japan described the perinatal period for two women with HAE and reviewed reports of 17 pregnancies to shed light on clinical characteristics and treatment strategies. Two of the study authors have received lecture fees from several companies, including CSL Behring, which markets Berinert. The treatment is approved in Japan for on-demand HAE treatment, as well as a short-term preventive therapy before a procedure.
The first case involved a 24-year-old woman who was diagnosed with HAE type 1 in her early twenties. Before her first pregnancy, she had a history of abdominal pain and peripheral swelling attacks in her hands and feet that occurred about once a year during the premenstrual period.
During pregnancy, however, her HAE activity increased markedly, with 23 total swelling attacks, mostly abdominal and peripheral, leading to 15 hospitalizations. All acute attacks were treated with Berinert, resulting in symptom resolution within one to two hours.
Because newer preventive HAE medications like Orladeyo (berotralstat) and Takhzyro (lanadelumab) were not available in Japan at that time, she elected to continue with tranexamic acid.
To minimize delivery-related risks, she received Berinert, administered one hour before delivery. At nine months, she delivered a healthy infant via vaginal birth. During the postpartum period, she had about one swelling attack per week, which was managed with Berinert.
Berinert used for on-demand treatment in about half of pregnancies
In the second case, a 35-year-old woman was diagnosed with HAE type 1 after her family was screened following her sister’s diagnosis. She experienced mild lip swelling about once a year.
She underwent three pregnancies over seven years, during which she received short-term preventive therapy with Berinert before all deliveries. Unlike the first case, she didn’t experience more swelling attacks during pregnancy, delivery, or postpartum.
No long-term preventive treatment was used because of her low initial attack rate, personal preference, and stable disease. She experienced only one mild lip swelling episode three years after the first birth, and none after the latter two.
Our integrated analysis demonstrates sustained reliance on [Berinert]-based [short-term preventive treatment], even amid expanding therapeutic options.
Among the 17 pregnancies reviewed, about half (52.9%) of women experienced more attacks during gestation, particularly in the early and late trimesters. In comparison, attack frequency remained unchanged in about one-third (35.3%) of pregnancies and decreased in one (5.9%).
“These findings parallel European data (59.2% increased), supporting the concept that hormonal changes — particularly in the first and third trimesters — can exacerbate HAE activity,” the researchers wrote.
Berinert was used for on-demand treatment in about half of pregnancies (52.6%), and as a short-term preventive treatment before delivery in most documented cases (80.0%). During the postpartum period, swelling attacks occurred in about one-third of cases (37.5%).
“Invasive procedures such as forceps delivery or cesarean section are recognized attack triggers, making pre-delivery [short-term preventive treatment] essential,” the team noted. “Our integrated analysis demonstrates sustained reliance on [Berinert]-based [short-term preventive treatment], even amid expanding therapeutic options,” the researchers wrote. It also “provides robust, context-specific evidence to support the development of structured, patient-centered perinatal HAE care pathways in Japan.”
About the Author
