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Pregnancy in Patients With Hereditary Angioedema and Normal C1 Inhibitor.
Natalia Gabriel1, Fernanda Marcelino2, Mariana P L Ferriani3
1Clinical Immunology, Faculdade de Medicina, Centro Universitario FMABC, Santo André, Brazil.
Frontiers in Allergy
|April 7, 2022
Summary
Pregnancy in women with hereditary angioedema with normal C1 inhibitor (HAE-nC1-INH) showed a similar abortion rate to the general population. However, HAE-nC1-INH symptoms were most pronounced during the first trimester, suggesting pregnancy may exacerbate the condition.
Area of Science:
- Immunology
- Genetics
- Obstetrics & Gynecology
Background:
- Hereditary angioedema with normal C1 inhibitor (HAE-nC1-INH) is a bradykinin-mediated angioedema where estrogens are a key trigger.
- While pregnancy outcomes in HAE with C1 inhibitor deficiency are documented, data for HAE-nC1-INH is limited.
Purpose of the Study:
- To evaluate the course and impact of pregnancy in women diagnosed with HAE-nC1-INH.
- To assess the frequency and timing of HAE attacks during pregnancy in this patient group.
Main Methods:
- Inclusion of women diagnosed with HAE-nC1-INH based on clinical, biochemical, and family history criteria.
- Application of a pregnancy-specific questionnaire and genetic evaluation for known mutations.
Main Results:
- 45 pregnancies in 26 HAE-nC1-INH patients were analyzed, with 17.8% spontaneous abortions, similar to the general population.
- HAE attacks occurred in 64.7% of pregnancies, predominantly in the 1st trimester (41.7%).
- Among patients with pre-pregnancy attacks, 9/15 experienced worsening symptoms during pregnancy.
Conclusions:
- Pregnancy outcomes in HAE-nC1-INH appear comparable to unaffected women regarding abortion rates.
- The first trimester poses a higher symptomatic burden for HAE-nC1-INH patients.
- Given the role of estrogens, pregnancy may potentially worsen the disease course in HAE-nC1-INH.
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