Treatment of Hereditary C1 Inhibitor Deficiency in Pregnancy
When a patient with hereditary C1 inhibitor deficiency is pregnant or breast-feeding, standard therapeutic options must be re-evaluated — both for efficacy and for safety to the developing fetus or nursing infant.
This protocol addresses pregnancy and lactation (breast-feeding) as the specific clinical context. During these states, C1INH is the recommended medication for use as either on-demand or prophylactic therapy.
The preferred approach centres on plasma-derived C1 inhibitor, with distinct guidance applying in pregnancy versus breast-feeding, and specific medication classes that are contraindicated in one or both settings — the full selection criteria are in the complete protocol.
References
DOI: 10.1016/j.jaip.2020.08.046
- During pregnancy and breast-feeding, C1INH is the recommended HAE medication for use as either on-demand or prophylactic therapy.
- C1INH is the preferred treatment in pregnancy due to its safety during pregnancy documented by several case reports and observational studies.
- We recommend pdC1INH or rhC1INH for on-demand or prophylaxis treatment during lactation.
- Anabolic androgens are specifically contraindicated during pregnancy as they cross the placenta and may result in virilization of the fetus.
- Anabolic androgens and tranexamic acid are secreted into the breast milk and should not be used during breast feeding.
View source ↗