Background/Epidemiology
- Rare, inflammatory vasculitis predominantly affecting the aorta and its major branches (occasionally pulmonary arteries)
- Characterised by granulomatous inflammation of the vessel wall, leading to vessel occlusion or weakness of the vessel wall
- Age of onset is usually 25-30; much more common in females (8:1); more common in Eastern Asia
Presentation
- Around 40-50% patients present with systemic features e.g. fever, fatigue, weight loss, arthralgia/myalgia
- Around 50-60% of patients present with ischaemia (or consequences of ischaemia) of some sort (occlusive disease)
- Claudication of the jaw/periphery; hypertension; syncope
- TIA/Stroke; seizures; dizziness/headaches
- Angina/MI, heart failure; aortic incompetence; aortic dissection; aortic coarctation
- Haemoptysis/pleuritis
- A classical finding is a >10mmHg discrepency between right and left arms. Other findings include absent/weak radial and peripheral (leg) pulses; arterial bruits; hypertension’
Investigations
- FBC may show a normocytic, normochromic anaemia
- Angiography is the main investigation- reveals features of aortic disease
Management
- High-dose steroids and immunosuppression
Notes on Medicine/Surgery 