If left untreated, hereditary angioedema (HAE) attacks gradually worsen over 12 to 24 hours and typically subside in 1 to 5 days. Treatment for HAE should be individualised to your patient’s needs and lifestyle to help reduce the burden of disease by preventing or attenuating attacks.1,2
According to the 2021 International WAO/EAACI Treatment
Guideline, HAE can be managed with:
WAO/EAACI recommendations3:
"We recommend that all attacks are:
- Considered for on-demand treatment. We recommend that any attack affecting or potentially affecting the upper airway is treated.
- Treated as early as possible.
- Treated with either C1-INH, ecallantide*, or icatibant."
"We recommend that all patients have sufficient medication for on-demand treatment of at least 2 attacks and carry on-demand medication at all times."
WAO/EAACI recommendations3:
"We recommend that all attacks are:
- Considered for on-demand treatment. We recommend that any attack affecting or potentially affecting the upper airway is treated.
- Treated as early as possible.
- Treated with either C1-INH, ecallantide*, or icatibant."
"We recommend that all patients have sufficient medication for on-demand treatment of at least 2 attacks and carry on-demand medication at all times."
-
WAO/EAACI recommendations3:
"We recommend the use of intravenous plasma-derived C1 inhibitor as first-line short-term prophylaxis."
“We recommend considering short-term prophylaxis before medical, surgical or dental procedures as well as exposure to other angioedema attack-inducing events."
"We suggest considering prophylaxis prior to exposure to patient-specific angioedema-inducing situations."
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WAO/EAACI recommendations3:
"We recommend the use of intravenous plasma-derived C1 inhibitor as first-line short-term prophylaxis."
“We recommend considering short-term prophylaxis before medical, surgical or dental procedures as well as exposure to other angioedema attack-inducing events."
"We suggest considering prophylaxis prior to exposure to patient-specific angioedema-inducing situations."
-
WAO/EAACI recommendation3:
“We recommend that patients are evaluated for long-term prophylaxis at every visit. Disease activity, burden, control, and patient preference should be taken into consideration. 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.""We recommend the use of lanadelumab, berotralstat, and plasma-derived C1-inhibitor as first-line, long-term prophylaxis."
-
WAO/EAACI recommendation3:
“We recommend that patients are evaluated for long-term prophylaxis at every visit. Disease activity, burden, control, and patient preference should be taken into consideration. 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.""We recommend the use of lanadelumab, berotralstat, and plasma-derived C1-inhibitor as first-line, long-term prophylaxis."
Availability of therapies differs by country.
*Only available in the US and a few Latin American countries.
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.4