Considerations in the management of hereditary angioedema due to C1-INH deficiency in women of childbearing age

Abstract

Abstract Hereditary angioedema (HAE) is a rare, autosomal disorder that manifests with unpredictable episodes of severe swelling of the skin and mucous membranes. These attacks can be highly disfiguring and range in severity from mild to—in cases of airway swelling—life-threatening. Fluctuations in female sex hormones—such as the changes that occur during puberty, menses, contraceptive use, pregnancy, and menopause—can all affect the frequency and severity of HAE attacks. Disease management decisions for women of childbearing age may be more complex and require additional considerations since they could develop complications related to contraception, pregnancy, labor, delivery, and lactation. In addition, some HAE treatment options are contraindicated during pregnancy. Discussions about medications used to treat HAE should include a risk–benefit assessment of the woman’s health status, her preferences, and other factors that are relevant to the choice of therapy. Planning prophylactic therapies that are effective and safe before, during, and after pregnancy can prevent gaps in treatment, ensure continuity of care, and reduce both disease burden and risk of adverse fetal outcomes. The 2020 US Hereditary Angioedema Association (HAEA) Medical Advisory Board and 2021 World Allergy Organization/European Academy of Allergy and Immunology (WAO/EAACI) Guidelines outline key considerations for managing HAE in females of childbearing age (15–45 years), with the goal of improving treatment efficacy and safety for this cohort of patients. Treatment decisions made in a collaborative manner involving the patient, HAE specialist and obstetric/gynecologic specialist, is the best approach to ensure optimal HAE management and safety in this patient population.