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Hereditary angioedema (HAE) is a rare, genetic disease that causes recurrent, unpredictable, and potentially life-threatening attacks of swelling in the body.1-3

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What is HAE?

Hereditary angioedema (HAE) is a rare disease that affects about 1 in 50,000 people in the entire world. It’s characterized by recurrent attacks of angioedema without urticaria or pruritis. HAE attacks, which may be painful and functionally disabling, can occur in the face, hands, feet, abdomen, genitals—and larynx, where attacks can be life-threatening. These attacks are largely unpredictable as they are caused by a C1 esterase inhibitor (C1-INH) deficiency or dysfunction. This lack of C1-INH leads to an increase in kallikrein activity, resulting in an excess of bradykinin production that produces HAE attacks.1,3,4

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How can HAE impact patients?

Hereditary angioedema (HAE) can impact the personal, social, and professional lives of patients and may hinder their ability to perform basic daily tasks, participate in social or professional events, travel, or even leave the house during HAE attacks.5

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How can HAE be managed?

Collaboration between knowledgeable healthcare providers and patients to ensure shared goals of treatment outcomes is important when developing an HAE management plan.4

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  1. Longhurst HJ, Bork K. Hereditary angioedema: causes, manifestations and treatment. Br J Hosp Med (Lond). 2006;67(12):654-657.
  2. Bork K, Meng G, Staubach P, Hardt J. Hereditary angioedema: new findings concerning symptoms, affected organs, and course. Am J Med. 2006;119(3):267-274.
  3. Zuraw BL. Clinical practice. Hereditary angioedema. N Engl J Med. 2008;359(10):1027-1036
  4. Busse PJ, Christiansen SC, Riedl MA, et al. US HAEA Medical Advisory Board 2020 guidelines for the management of hereditary angioedema. J Allergy Clin Immunol Pract. 2021;9(1):132-150. doi:10.1016/j.jaip.2020.08.046
  5. Longhurst HJ, Bork K. Hereditary angioedema: an update on causes, manifestations and treatment. Br J Hosp Med (Lond). 2019;80(7):391-398. doi:10.12968/hmed.2019.80.7.391