USE OF C1 ESTERASE INHIBITORS (C1-INH) IN PREGNANCY

C1 esterase inhibitors (C1-INH) are endogenous protease inhibitors belonging to the serpin superfamily of proteins. Exogenous C1 esterase inhibitors, derived from plasma, are used to treat hereditary angioedema (HAE), a condition characterised by a deficiency of this protein.

The available published data concerning the safety of C1 esterase inhibitor use in pregnancy are limited to a small number of case series and case reports, many of which focus on the efficacy of treatment rather than pregnancy outcome. Fetal outcomes have been reported after documented first trimester exposure in 18 pregnancies, with no congenital malformations noted. Exposure in pregnancy has not shown any adverse fetal or neonatal effects attributable to C1 esterase inhibitor, but as experience of use is still extremely limited, a risk of adverse fetal effects cannot be excluded.

Although data on C1 esterase inhibitor use during pregnancy are limited, due to the potential severity of HAE, consensus guidelines advise use as the first line treatment for acute attacks during pregnancy. Severe cases may also require regular C1 INH replacement therapy. Pre-delivery prophylaxis with C1 INH and the availability of C1 INH in the delivery suite should be considered where appropriate.

Exposure to C1 esterase inhibitors at any stage of pregnancy would not usually be regarded as medical grounds for termination of pregnancy or any additional fetal monitoring. However, other risk factors may be present in individual cases which may independently increase the risk of adverse pregnancy outcome. Clinicians are reminded of the importance of consideration of such factors when performing case-specific risk assessments.