Hereditary angioedema (HAE) is a rare disease caused by a C1 inhibitor (C1-INH) deficit. Clinically, HAE is manifested by repeated episodes of localized subcutaneous or submucosal oedema attacks. Managing HAE patients in pregnancy is challenging, since there are only limited data on the safety and efficacy of various therapeutic approaches.
We present our clinical experience treating acute HAE attacks during pregnancy in six consecutive patients.
During the pregnancies, 79 HAE attacks occurred. The most frequent were abdominal 53 (67.1%) followed by peripheral 21 (26.6%), facial 10 (12.7%), and laryngeal 10 (12.7%) oedemas; 13 (16.5%) attacks were combined. Fifty (63.3%) attacks were treated with recombinant human C1-INH (rhC1-INH); 17 (21.5%) with plasma-derived, pasteurized, nanofiltered C1-INH (pnfC1-INH); 13 (16.5%) with icatibant; and 1 (1.3%) with plasma-derived, nanofiltered C1-INH (nfC1-INH). Treatment had to be repeated in 5 attacks (6.3%). All six deliveries (one caesarean section and five spontaneous vaginal deliveries) were complication free. All pregnancies went to the full term and the patients delivered healthy babies with a birth weight ranging from 2850 to 3690 g. No congenital abnormalities were detected in the neonates. No abortions occurred.
Our results show good C1-INH or icatibant treatment efficacy for HAE attacks in pregnancy. The treatment by the first drug used was effective in 93.7% of all attacks. In 6.3% of attacks, a second treatment had to be used. No adverse effects were observed.
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This report was supported by MUNI/A/0925/2017 grant from the Ministry of Education, Youth and Sports of the Czech Republic and by grants 15-28732A, 16-34414A, and NV18-05-00330 from the Czech Ministry of Health.
Conflict of Interest
Roman Hakl declares the following real or perceived conflicts of interest: Roman Hakl has received a travel grant from Pharming Group NV, CSL Behring and Shire.
Pavel Kuklínek has no conflict of interest.
Irena Krčmová has no conflict of interest.
Pavlína Králíčková has no conflict of interest.
Tomáš Freiberger has no conflict of interest.
Petr Janků has no conflict of interest.
Marcela Vlková has no conflict of interest.
Jiří Litzman has no conflict of interest.
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Hakl, R., Kuklínek, P., Krčmová, I. et al. Treatment of Hereditary Angioedema Attacks with Icatibant and Recombinant C1 Inhibitor During Pregnancy. J Clin Immunol 38, 810–815 (2018). https://doi.org/10.1007/s10875-018-0553-4
- Hereditary angioedema
- recombinant C1 inhibitor