Hereditary Angioedema (HAE)

Hereditary angioedema (HAE) is an inherited disorder. Symptoms include:

  • Recurrent swelling in the arms, legs, abdomen, genitalia or face that is not usually painful but can be severe
  • Painful swelling of the bowel wall (the lining of the intestines) associated with nausea and vomiting
  • Swelling in the throat that can cause respiratory distress

The most common cause of HAE is a decrease in C1 esterase inhibitor (C1 INH), a protein that is part of our body’s normal immune system. A peptide called bradykinin is thought to be responsible for the HAE symptoms of swelling, inflammation and pain.

Patients with HAE do not experience hives.

Pressure, trauma, or emotional stress can trigger an attack; however, the trigger of most attacks is unknown.

  • Typical attacks last about 1-3 days.
  • If your child develops an acute attack, contact your physician or go to the emergency room.

New medications have been developed to help treat acute attacks for HAE. 

  • Cinryze and Berinert work by blocking bradykinin and are used to treat acute facial and abdominal attacks in patients 12 years and older.
  • Kalbitor, which contains a plasma kallikrein inhibitor, is used to treat attacks of hereditary angioedema in patients who are 12 years and older.
  • Fresh frozen plasma may also be used to treat acute attacks.
  • Danazol, a type of steroid, is useful for the prevention of an acute attack.
  • Cinryze, a new medication that contains C1 INH, can be used to prevent an attack in patients 18 years or older.
  • Fresh frozen plasma has been used successfully to prevent an attack prior to surgery or a dental procedure.  

Clinical trials investigating new medications for the treatment of HAE are ongoing through the University of Cincinnati.

Last Updated 07/2015