HAE a refresher

HAE arises from a deficiency in functional C1 esterase inhibitor (C1-INH), which can have potentially life threatening implications.1,2

HAE has an incidence of
1:50,000 worldwide¹

HAE is caused by a C1 esterase
inhibitor (C1-INH) deficiency due to autosomal dominant or de novo mutation(s) in the Serpin Family G Member 1 (SERPING1) gene1,3

HAE attacks involve recurrent
episodes of subcutaneous/
submucosal swelling that can
affect multiple parts of the body2,4

Disease Burden

The unpredictability, frequency, and potentially life threatening nature of HAE attacks have a negative impact on all aspects of life5

Did you know?

43% of patients with HAE exhibit depressive symptoms5

59% of patients miss leisure days, thus HAE can impact work/school, social, and family life5

64% of HAE attacks are caused by trigger events e.g., emotional distress, infection, and injury4

Unmet need

Patients need confidence in their HAE treatment

Patients with HAE can find the risk of breakthrough attacks of prophylactic treatments stressful as repeat doses of rescue medicine may be needed5-8

Knowing that their treatment may not work in one dose can be stressful for patients. Therefore, on demand treatment that works first time is necessary for patient’s quality of life5

Treatment Guidelines

What do the World Allergy Organisation (WAO) and European Academy of Allergy and Clinical Immunology (EAACI) guidelines recommend?9

All attacks should be treated as early as possible and with on demand treatment if patients are eligible⁹

Patients should have on demand treatment at home for ≥2 attacks and should always carry on demand medication

Treatment aims to achieve total control of disease and normalise patient lives


  1. Cancian M, et al. Pediatr Allergy Immunol 2020;31 Suppl 24:22-24.
  2. European Value Dossier: RUCONEST® in the management of Acute Hereditary Angioedema. IQVIA, 2018.
  3. Piñero-Saavedra M, et al. Rare Dis Res Treat 2017;2(4):14-19.
  4. Caballero T, et al. J Investig Allergol Clin Immunol 2016;26(6):383-386.
  5. Busse PJ, et al. J Allergy Clin Immunol Pract 2021;9(1):132-150.e3.
  6. Riedl MA, et al. Ann Allergy Asthma Immunol 2012;108(1):49-53.
  7. Banerji A, et al. JAMA 2018;320(20):2108-2121.
  8. Malbran A, et al. Clin Exp Immunol 2014;177:544-553.
  9. Maurer M, et al. Allergy 2022;00:1-30.