Earlier this year, the European Journal of Allergy and Clinical Immunology published the 2021 revision and update of the international WAO/EAACI guideline for the management of HAE. 

The updated guideline reflects consensus among an international panel of 60 HAE experts, and includes 28 recommendations that provide physicians and patients with guidance on important clinical issues, such as: (1) How should HAE be diagnosed? (2) When should HAE patients receive prophylactic on top of on-demand treatment, and what treatments should be used? (3) What are the goals of treatment? (4) Should HAE management be different for special HAE patient groups such as children or pregnant/breastfeeding women? and (5) How should HAE patients monitor their disease activity, impact, and control?

These are the 28 recommendations:

  1. We recommend that all patients suspected to have HAE are assessed for blood levels of C1-INH function, C1-INH protein, and C4
  2. We suggest that testing for C1-INH function, C1-INH protein, and C4 is repeated in patients who test positive, to confirm the diagnosis of HAE-1/2
  3. We recommend that patients who are suspected to have HAE and have normal C1-INH levels and function are assessed for known mutations underlying HAE-nC1-INH
  4. We recommend that all attacks are considered for on-demand treatment
  5. We recommend that any attack affecting or potentially affecting the upper airway is treated
  6. We recommend that attacks are treated as early as possible
  7. We recommend that attacks are treated with either intravenous C1 inhibitor, ecallantide or icatibant
  8. We recommend that intubation or surgical airway intervention is considered early in progressive upper airway edema
  9. We recommend that all patients have sufficient medication for on-demand treatment of at least two attacks and carry on-demand medication at all times
  10. We recommend considering short-term prophylaxis before medical, surgical or dental procedures as well as exposure to other angioedema attack-inducing events
  11. We recommend the use of intravenous plasma-derived C1 inhibitor as first-line short-term prophylaxis
  12. We suggest considering prophylaxis prior to exposure to patient-specific angioedema-inducing situations
  13. We recommend that the goals of treatment are to achieve total control of the disease and to normalize patients’ lives
  14. We recommend that patients are evaluated for long-term prophylaxis at every visit, taking disease activity, burden, and control as well as patient preference into consideration
  15. We recommend the use of plasma-derived C1 inhibitor as first-line long-term prophylaxis
  16. We recommend the use of lanadelumab as first-line long-term prophylaxis
  17. We recommend the use of berotralstat as first-line long-term prophylaxis
  18. We recommend the use of androgens only as second-line long-term prophylaxis
  19. We suggest all patients who are using long-term prophylaxis be routinely monitored for disease activity, impact, and control to inform optimization of treatment dosages and outcomes
  20. We recommend testing children from HAE-affected families be carried out as soon as possible and all offspring of an affected parent be tested
  21. We recommend C1 inhibitor or icatibant be used for the treatment of attacks in children under the age of 12
  22. We recommend plasma-derived C1 inhibitor as the preferred therapy during pregnancy and lactation
  23. We recommend that all patients have an action plan
  24. We recommend that HAE-specific comprehensive, integrated care is available for all patients
  25. We recommend that patients are treated by a specialist with specific expertise in managing HAE
  26. We recommend that all patients who are provided with on-demand treatment licensed for self-administration should be taught to self-administer
  27. We recommend that all patients should be educated about triggers that may induce attacks
  28. We recommend screening family members of patients for HAE

HAEi President & CEO Anthony J. Castaldo and EVP & COO Henrik Balle Boysen represented the global patient community as authors of the guidelines.

The guideline can be accessed online at onlinelibrary.wiley.com/doi/10.1111/all.15214