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Treatment of Hereditary Angioedema Attacks with Icatibant and Recombinant C1 Inhibitor During Pregnancy

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Abstract

Purpose

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.

Methods

We present our clinical experience treating acute HAE attacks during pregnancy in six consecutive patients.

Results

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.

Conclusions

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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Funding

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.

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Authors and Affiliations

  1. Department of Clinical Immunology and Allergology, St. Anne’s University Hospital in Brno, Brno, Czech Republic

    Roman Hakl, Pavel Kuklínek, Tomáš Freiberger, Marcela Vlková & Jiří Litzman

  2. Faculty of Medicine, Masaryk University, Brno, Czech Republic

    Roman Hakl, Tomáš Freiberger, Marcela Vlková & Jiří Litzman

  3. Department of Clinical Immunology and Allergy, University Hospital, Hradec Kralove, Czech Republic

    Irena Krčmová & Pavlína Králíčková

  4. Centre for Cardiovascular Surgery and Transplantation, Brno, Czech Republic

    Tomáš Freiberger

  5. Department of Obstetrics and Gynecology, University Hospital Brno, Brno, Czech Republic

    Petr Janků

Authors
  1. Roman Hakl
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  2. Pavel Kuklínek
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  3. Irena Krčmová
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  7. Marcela Vlková
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  8. Jiří Litzman
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Corresponding author

Correspondence to Roman Hakl.

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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

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  • DOI: https://doi.org/10.1007/s10875-018-0553-4

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