29th January 2024

Background

Epidemiology 

This is a rare disease with a 9:1 female predominance. It typically occurs in people aged between 15-45 years and is more commonly seen in those of Asian heritage.

Aetiology 

The cause is unknown, with evidence suggestive of infection of some kind (viral, bacterial, other) predisposing to the disease. 

Pathophysiology

Granulomatous inflammation of the aorta and its major branches occur. This results in thickening and stenosis of the involved blood vessels. The chronic inflammation then causes subsequent vascular symptoms.