Research
General gynecology
Disease expression in women with hereditary angioedema

https://doi.org/10.1016/j.ajog.2008.04.034Get rights and content

Objective

Fluctuations in sex hormones can trigger angioedema attacks in women with hereditary angioedema. Combined oral contraceptive therapies, as well as pregnancy, can induce severe attacks. The course of angioedema may be very variable in different women.

Study Design

Within the PREHAEAT project launched by the European Union, data on 150 postpubertal women with hereditary angioedema were collected in 8 countries, using a patient-based questionnaire.

Results

Puberty worsened the disease for 62%. Combined oral contraceptives worsened the disease for 79%, whereas progestogen-only pills improved it for 64%. During pregnancies, 38% of women had more attacks, but 30% had fewer attacks. Vaginal delivery was usually uncomplicated. Attacks occurred within 48 hours in only 6% of cases. Those more severely affected during menses had more symptoms during pregnancies, suggesting a hormone-sensitive phenotype for some patients.

Conclusion

The course of angioedema in women with C1 inhibitor deficiency is affected by physiologic hormonal changes; consequently, physicians should take these into account when advising on management.

Section snippets

Materials and Methods

A retrospective study was conducted. One hundred fifty postpubertal women were enrolled within the framework of the PREHAEAT Project (QLG1-CT-2002601359) of the European Union. The aim of this project was to improve the quality of life of patients with HAE. Hormonal problems in HAE were the subject of the Fifth Work Package of the Project. All women had a C1Inh functional level of 40% or lower, family or personal history of HAE, and/or mutation of the C1Inh gene.

A specific patient-based

Characteristics of the sample

Data of 150 postpubertal women from 8 countries: Hungary (32%), Germany (19%), Denmark (10%), France (10%), Spain (10%), Norway (8%), United Kingdom (6%), and Switzerland (5%), were analyzed. The average age of puberty was 13.5 years (range: 9-17 years). Forty-four women had undergone menopause at an average age of 48.5 years (range: 37-56 years). One hundred seven women had experienced 227 pregnancies.

Puberty and menstrual cycle: Influence on HAE course

Puberty worsened the disease for 62%; the remaining 38% percent of women reported no change.

Comment

This retrospective analysis answers frequently asked questions about puberty, fertility, pregnancies, and contraception in women with HAE. There is no increase in gynecologic events or infertility. Pregnancy is likely to worsen, improve, or to have no effect on disease severity equally. The observation4 that HAE symptoms improve during the 2 last trimesters was not confirmed in our study. Because of insufficient data, we could not answer the question whether HAE course during pregnancy is

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    This project was performed within the framework of the PREHAEAT project (QLG1-CT-2002-01359) of the European Union and was supported by grants from the thematic action 5 of the French 2002 Programme Hospitalier de Recherche Clinique PHRC.

    Cite this article as: Bouillet L, Longhurst H, Boccon-Gibod I, et al. Disease expression in women with hereditary angioedema. Am J Obstet Gynecol 2008;199:484.e1-484.e4.

    Reprints not available from the authors.

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