Takayasu arteritis

Discussion:

The above described findings are those of Takayasu arteritis with diffuse aortitis, pulmonary arteritis, diffuse tight stenosis of the left CCA and subclavian artery, as well as stenosis of the left and right pulmonary arteries with mild pulmonary hypertension.

Takayasu arteritis (pulseless disease) is a chronic, granulomatous, large-vessel vasculitis affecting predominantly the aorta and its main branches in young females <30 years. Intimal proliferation eventually ends up with stenosis and occlusions of the affected arteries. Arbitrary classification of Takayasu arteritis includes 4 types:

Type 1: aortic arch and its main branches.

Type 2: abdominal aorta.

Type 3: entire aorta.

Type 4: pulmonary arteries are involved as well with peripheral pruning.

The main differential diagnosis is Giant cell arteritis. It is a vasculitis of medium-sized arteries; common in older patients >50 years. Aortitis (mostly the ascending aorta) in 10% of cases. Diagnosis is via biopsy of the superficial temporal artery.

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