Triggers and short-term prophylaxis in patients with hereditary angioedema

Background:

Hereditary angioedema (HAE) is a rare disease that affects 1 in 60,000; however, despite being extremely rare, the severity of the disease can cause significant limitations to quality of life. In addition, attacks can be fatal and require urgent care.

Methods:

We searched PubMed and Google for Hereditary Angioedema and prophylaxis, short term prophylaxis, surgery, medical procedures, dental work, triggers.

Results:

The main triggers are estrogens, Angiotensin Converting Enzyme Inhibitors (ACI) inhibitors, trauma, dental work, stress, surgery, manipulation of the upper airway, and medical procedures. Prophylaxis is often used long term to prevent attacks; before known triggers, prophylaxis is referred to as short-term prophylaxis (STP). When to initiate STP, what to use, and what dose to use have not been adequately researched, but there is consensus that, whenever the upper airway is manipulated, STP is essential. In addition, consensus has been reached that an IV C1 inhibitor is the preferred STP agent, and it is my opinion that dosing at 20 units/kg allows dosing for all ages and also allows average-size adults to receive >1000 units because failures at 1000 units have been documented in the literature.

Conclusions:

This article focused on triggers and preprocedural STP and not on pre-event STP, which is often used before important life events; however, medications and dosing are the same for pre-event prophylaxis.