creating a treatment plan to manage hereditary angioedema

 

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

 

2017 World Allergy Organization Treatment Guideline

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 C1-INH or icatibant (a bradykinin receptor antagonist). Ecallantide (kallikrein inhibitor) can be used to treat attacks but is only approved in the United States1
     
  • All patients should have on-demand treatment for 2 attacks, to be carried with them 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 C1-INH or icatibant (a bradykinin receptor antagonist). Ecallantide (kallikrein inhibitor) can be used to treat attacks but is only approved in the United States1
     
  • All patients should have on-demand treatment for 2 attacks, to be carried with them 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. Preprocedural prophylaxis with C1-INH concentrate is therefore recommended for all medical, surgical, and dental procedures associated with any mechanical impact to the upper aerodigestive tract.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 burden and patient preference.1

 

Dosage and treatment interval should be adapted as needed to minimise burden of disease.1

imagegood to know
Androgens are 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.
  • 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. Preprocedural prophylaxis with C1-INH concentrate is therefore recommended for all medical, surgical, and dental procedures associated with any mechanical impact to the upper aerodigestive tract.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 burden and patient preference.1

 

Dosage and treatment interval should be adapted as needed to minimise burden of disease.1

imagegood to know
Androgens are 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.

Availability of therapies differs by country.

Watch and learn:
New Views on Prevention & On-Demand Therapy: Highlights from the 2017 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

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