Hereditary Angioedema

  • Bruce L. ZurawEmail author
  • Sandra C. Christiansen


Hereditary angioedema comprises a group of rare autosomal dominant disorders that manifest as recurrent attacks of swelling with attendant morbidity and mortality. HAE can occur with or without C1 inhibitor deficiency with each category comprised of additional subtypes. The underlying pathophysiology is generation of bradykinin with ensuing vascular leak and angioedema. HAE usually presents in childhood often with substantial delay until establishing the correct diagnosis, which is critically important to guide appropriate treatment. The management of HAE in women presents distinct challenges. Hormonal replacement, contraception, and pregnancy often escalate attack frequency and severity. Treatment of HAE during pregnancy provides additional complexities should the disease intensify as many of the potential therapies are not recognized as safe. This chapter reviews the current classification of HAE, impact of sex hormones, the relationship between pregnancy and severity of swelling, and the management of HAE during pregnancy and breastfeeding. Through gaining an understanding of disease mechanisms and the unique issues facing women, the clinician will be able to successfully manage HAE patients during pregnancy.


HAE C1 inhibitor Estrogen Bradykinin Sex hormones Pregnancy 


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© Springer Nature Switzerland AG 2019

Authors and Affiliations

  1. 1.Department of MedicineUniversity of California San DiegoLa JollaUSA
  2. 2.San Diego VA HealthcareLa JollaUSA

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