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.

Centre for Cardiovascular Surgery and Transplantation Brno Czech Republic

Department of Clinical Immunology and Allergology St Anne's University Hospital in Brno Brno Czech Republic

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

Department of Obstetrics and Gynecology University Hospital Brno Brno Czech Republic

Faculty of Medicine Masaryk University Brno Czech Republic

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Eur J Obstet Gynecol Reprod Biol. 2012 Dec;165(2):366-7 PubMed

J Investig Allergol Clin Immunol. 2016;26(3):161-7 PubMed

Orphanet J Rare Dis. 2016 Apr 21;11:43 PubMed

Allergy. 2012 Feb;67(2):147-57 PubMed

J Investig Allergol Clin Immunol. 2017;27(5):322-323 PubMed

Am J Obstet Gynecol. 2010 Aug;203(2):131.e1-7 PubMed

Allergy Asthma Proc. 2013 Jan-Feb;34(1):13-8 PubMed

N Engl J Med. 2010 Aug 5;363(6):532-41 PubMed

PLoS One. 2013;8(2):e56712 PubMed

Allergy Asthma Immunol Res. 2017 Jan;9(1):96-98 PubMed

Aust N Z J Obstet Gynaecol. 2009 Feb;49(1):2-5 PubMed

J Investig Allergol Clin Immunol. 2015;25(6):447-9 PubMed

J Obstet Gynaecol Res. 2016 Aug;42(8):1026-8 PubMed

N Engl J Med. 2008 Sep 4;359(10):1027-36 PubMed

J Allergy Clin Immunol. 2012 Feb;129(2):308-20 PubMed

J Biol Chem. 1989 Feb 25;264(6):3066-71 PubMed

Allergy Asthma Clin Immunol. 2010 Jul 28;6(1):17 PubMed

Clin Endocrinol (Oxf). 2004 Apr;60(4):508-15 PubMed

Eur J Obstet Gynecol Reprod Biol. 2010 Sep;152(1):44-9 PubMed

Immunotherapy. 2015;7(7):739-52 PubMed

Clin Exp Allergy. 2014 Dec;44(12):1503-14 PubMed

Am J Med. 2006 Mar;119(3):267-74 PubMed