- an inflammatory and stenotic disease of medium and large-sized arteries characterized by strong predilection for the aortic arch and its branches
- a.k.a aortic arch syndrome
- 2-3 per million
- 80% are women and mean age of onset is 30 years
- panarteritis with inflammatory mononuclear cell infiltrates and occasionally giant cells
- presents with fever, night sweats, arthralgia, anorexia and weight loss
- pulses are commonly absent in the involved vessels, particularly subclavian artery
- hypertension occurs in 32-93% of patients and contribute to renal, cardiac and cerebral injury
- diagnosis is confirmed by the characteristic pattern on arteriography which includes irregular vessel walls, stenosis, post stenotic dilatation, aneurysm formation, occlusion and evidence of increased collateral circulation
- corticosteroid with the addition of steroid sparing agents such as methotrexate or azathioprine are mainstay of treatment
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