Takayasu Disease

Worldwide incidence: 2.6 cases per million per y.

Race with highest prevalence: Asian.

Females 8–9 times more likely to be affected than males.

Severe uncontrolled Htn leading to end-organ dysfunction

Stenosis of major blood vessels affecting regional circulation

Difficulties in monitoring BP.

Long-term corticosteroids.

Multiple occlusions of peripheral arteries, CHF, stroke, cardiac valve dysfunction, hypertensive episodes, intracranial hemorrhage, and iatrogenic adrenal suppression

A rare systemic inflammatory large-vessel vasculitis primarily affecting the aorta and its main branches

Initial “inflammatory phase” characterized by systemic illness with malaise, fever, weight loss, and fatigue

Secondary “pulseless phase” characterized by vascular insufficiency from intimal narrowing of the vessels manifesting as arm or leg claudication, renal artery stenosis causing Htn, and neurologic manifestations due to decreased blood flow to the brain

Unknown; some evidence to support a genetic predisposition.

Cell-mediated immune mechanisms are of primary importance.

Panarteritic inflammatory infiltrates cause marked thickening of the affected artery. Segmental and patchy granulomatous inflammation leads to arterial stenosis, thrombosis, and aneurysms.

Initial vascular lesions frequently occur in the left subclavian artery, with other branches and the aorta becoming affected as the disease progresses.