Pregnant Women with Hereditary Angioedema May Benefit from Long-term Treatment with Berinert, Report Suggests

Long-term treatment with human plasma-derived C1 inhibitor may help women with hereditary angioedema get pregnant and carry out the pregnancy to full term without risking complications, a case report suggests.

The study, from a hospital in Valencia, Spain, titled, “Successful long-term prophylaxis with human plasma-derived C1 inhibitor in planning and carrying out pregnancy,” was published in the journal Allergology International.

Hereditary angioedema (HAE) is caused by mutations in the C1 inhibitor (C1-INH) that reduce the protein’s activity. The disorder is characterized by recurrent attacks of edema in different locations of the body, which might be worsened during pregnancy due to increased estrogen levels.

In most cases, angioedema takes an unpredictable course in pregnant women, with the frequency and severity of angioedema attacks being highly variable between pregnancies even in the same woman, researchers explained.

Researchers reported the case of a 34-year-old women with HAE who was having difficulties in conceiving as a result of her therapy for the disease – she had long-term treatment with attenuated androgens before the disease showed symptoms.

While the use of attenuated androgens is on the decrease due to several side effects, treatment options for pregnant women are still limited.

“Attenuated androgens can induce amenorrhea [the absence of menstruation] and menstrual irregularities that may hinder conception planning, as in the case of our patient,” researchers wrote.

After discontinuing her treatment, the patient started on Berinert (human plasma-derived C1-INH), a type of therapy that “has been proven safe and effective in acute attacks, but also as short and long-term prophylaxis [preventive therapy].”

“After starting long-term prophylaxis with [Berinert] twice a week, she could achieve and carry out a successful pregnancy,” researchers wrote. The patient continued to self-administer the therapy throughout pregnancy without any complications arising.

Previously, a 34-year-old woman with type 1 HAE, also undergoing treatment with attenuated androgens (danazol) was also treated with Berinert after her first and during her second pregnancy. Delivery went smoothly and the patient maintained adequate control of the disease.

Although recent, several studies have reported the use of Berinert not only for the treatment of HAE acute attacks, but also as short-term preventive treatment, and long-term prophylaxis during pregnancy.

In fact, “C1-INH was found to be the safest option for prophylactic and acute treatment as an on demand treatment in pregnant patients with C1-INH-HAE in a retrospective analysis with a total of 16 subjects,” researchers wrote.

Overall, “long-term prophylaxis with [Berinert] seems to be a good alternative to accomplish and carry out pregnancy in women with C1-INH-HAE at a reproductive age,” the study concluded.