All patients with hereditary angioedema (HAE) should have an individualised treatment plan that is carefully developed to fit their needs and lifestyle. Individualised treatment plans should address preventive measures, home care, and self-administration, as well as an effective emergency (on-demand) treatment plan with clear instructions on how best to use medications to treat HAE attacks.1
2021 International WAO/EAACI Guideline for Management of HAE
It is recommended that all patients with HAE should be educated about possible triggers which may induce HAE attacks.1
- All attacks should be considered for on-demand treatment and treated as early as possible. Any attack potentially affecting the upper airway should be treated1
- Attacks can be treated with either intravenous C1 inhibitor, ecallanatide or icatibant. Ecallantide (kallikrein inhibitor) can be used to treat attacks but is only approved in the United States and a few Latin American countries1
- All patients should have sufficient medication for on-demand treatment of at least 2 attacks and carry on-demand medication at all times1
- All attacks should be considered for on-demand treatment and treated as early as possible. Any attack potentially affecting the upper airway should be treated1
- Attacks can be treated with either intravenous C1 inhibitor, ecallanatide or icatibant. Ecallantide (kallikrein inhibitor) can be used to treat attacks but is only approved in the United States and a few Latin American countries1
- All patients should have sufficient medication for on-demand treatment of at least 2 attacks and carry on-demand medication at all times1
- Short-term prophylaxis: Case reports and series suggest that despite prophylaxis, swellings may occur even after relatively minor procedures. However, several reports document a reduction in the incidence of swelling for both adults and children with preprocedural prophylaxis, and the response appears to be dose related. Short-term prophylaxis before medical, surgical or dental procedures as well as exposure to other angioedema attack-inducing events is therefore recommended.1
- Long-term prophylaxis: Consider for patients who face events in life that are associated with increased disease activity; patients should be evaluated for long-term prophylaxis at every visit, taking into account disease activity, burden, control, and patient preference. The goals of treatment are to achieve total control of the disease and to normalize patients' lives, which can currently only be achieved by long-term prophylaxis.1
- Lanadelumab, berotralstat, and plasma-derived C1-inhibitor (C1-INH) are recommended as first-line, long-term prophylaxis.1
good to knowAndrogens are only recommended as second-line treatment, with C1-INH as first line. Androgens must be regarded critically, especially in light of their adverse androgenic and anabolic effects, drug interactions, and contraindications.1
- Short-term prophylaxis: Case reports and series suggest that despite prophylaxis, swellings may occur even after relatively minor procedures. However, several reports document a reduction in the incidence of swelling for both adults and children with preprocedural prophylaxis, and the response appears to be dose related. Short-term prophylaxis before medical, surgical or dental procedures as well as exposure to other angioedema attack-inducing events is therefore recommended.1
- Long-term prophylaxis: Consider for patients who face events in life that are associated with increased disease activity; patients should be evaluated for long-term prophylaxis at every visit, taking into account disease activity, burden, control, and patient preference. The goals of treatment are to achieve total control of the disease and to normalize patients' lives, which can currently only be achieved by long-term prophylaxis.1
- Lanadelumab, berotralstat, and plasma-derived C1-inhibitor (C1-INH) are recommended as first-line, long-term prophylaxis.1
good to knowAndrogens are only recommended as second-line treatment, with C1-INH as first line. Androgens must be regarded critically, especially in light of their adverse androgenic and anabolic effects, drug interactions, and contraindications.1
Availability of therapies differs by country.
Watch and learn:
A New View on Prevention & On-Demand Therapy: Highlights from the 2021 International WAO/EAACI Guideline
Watch Dr Marcus Maurer review some updates to the treatment guideline.
Please note that current treatment guidelines are for patients with Type 1 and Type 2 HAE only; the pathogenesis of other forms of HAE is not well-characterised, and therefore, therapeutic options may not be similarly effective.1