Successful treatment with Cinryze® replacement therapy of a pregnant patient with hereditary angioedema: a case report

Abstract

Background Hereditary angioedema (HAE) is a rare disease characterized with recurrent swelling of subcutaneous or mucosal tissue that resolves in approximately 3 days. It can be presented with peripheral edema, abdominal and life-threatening laryngeal angioedema. A variety of triggers are known to cause episodes of angioedema including estrogen exposure. There are different reports regarding the effect of pregnancy on HAE attacks, and in some patients, the pregnancy is a recognized triggering factor. Case presentation We present a female Caucasian patient with pre-existing HAE and disease exacerbations during pregnancy, requiring prophylactic use of plasma-derived C1 inhibitor concentrate. She was treated with Cinryze® replacement therapy throughout the pregnancy 1000 IU i.v. 48 times. She gave birth to a healthy male infant, via C-section. After the delivery, the patient was symptom-free for 6 months and required no treatment for HAE. Conclusions In the case presented, the angioedema attacks worsened as the pregnancy progressed. The treatment with Cinryze® replacement therapy was effective and safe during pregnancy, with no adverse effects on the infant.

The international WAO/EAACI guideline for the management of hereditary angioedema—The 2021 revision and update

“…C1-INH is recommended as first-line therapy for pregnant or breastfeeding HAE-1/2 patients as it is safe and effective (Recommendation 22). 222,[325][326][327][328][329] The use of ecallantide, lanadelumab and berotralstat in pregnancy is off label and not recommended as no published experience is available as of now. Although contraindicated by label, there are isolated case reports about the administration of icatibant during pregnancy with no maternal or fetal adverse effects reported.…”

“…C1‐INH is recommended as first‐line therapy for pregnant or breastfeeding HAE‐1/2 patients as it is safe and effective (Recommendation 22) 222,325–329 …”

The international WAO/EAACI guideline for the management of hereditary angioedema – The 2021 revision and update

“…C1–INH is recommended as first-line therapy for pregnant or breast-feeding HAE-1/2 patients as it is safe and effective (Recommendation 22). 222 , 325 , 326 , 327 , 328 , 329 The use of ecallantide, lanadelumab and berotralstat in pregnancy is off label and not recommended as no published experience is available as of now. Although contraindicated by label, there are isolated case reports about the administration of icatibant during pregnancy with no maternal or fetal adverse effects reported.…”

Long-term Prophylaxis with Androgens in the management of Hereditary Angioedema (HAE) in emerging countries

“…Because there are not enough safety data on the potential effects of androgens on bone maturation, growth, and development, use of attenuated androgens is not recommended in children [51]. Moreover, pregnant females should not use androgen therapy because of adverse effects, while prophylactic use of plasma-derived C1 inhibitor concentrate was found to be effective in some cases [52,53]. Therefore, patients with HAE who are administered androgens long term should have regular physical examinations to adjust their medication regimen [1,31].…”