50% of HAE patients have laryngeal swellings that are potentially fatal despite prophylaxis

Angioedema (AE) Classification (click to enlarge the image):

Here are some excerpts from the 2012 Lancet review of Hereditary angio-oedema (spelled angioedema in the U.S., HAE):

Hereditary angioedema is caused by a heterozygous deficiency of C1 inhibitor. It regulates inflammatory pathways.

Patients with HAE have episodic skin or mucosal swellings because of a failure to control local production of bradykinin. Swellings typically evolve in several hours and persist for a few days.

In addition to orofacial angioedema, painless swellings affect the arms and legs, and causes disfigurement and interference with work and activities of daily living.

Angioedema may affect the GI tract cause severe pain often with vomiting due to edematous bowel obstruction.

2% of swellings involve the larynx and can be fatal if untreated.

50% of patients have laryngeal swellings - and they are potentially fatal, despite prophylaxis.

Several new treatments for HAE have been developed and launched on the market in the past 5 years.

The video "Ever Heard of HAE?" was produced by Shire Australia to help raise awareness of hereditary angioedema (HAE) among the general community and prompt those who may be living with HAE unknowingly, to seek help:


Hereditary angio-oedema. The Lancet,  Volume 379, Issue 9814, Pages 474 - 481, 4 February 2012.

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