Takayasu Vasculitis presenting as Subclavian Steal Syndrome - A case report

Abstract

Background: Takayasu arteritis (TAK) is a rare, systemic, immune mediated large-vessel vasculitis of unknown etiology that most commonly affects women of childbearing age. It is a granulomatous inflammation of the aorta and its major branches that leads to thickening, narrowing, occlusion, or dilation of the affected arteries. The subclavian steal syndrome occurs due to the reversal of flow in the vertebral artery toward the subclavian artery (during periods of increased demand) because of stenosis/occlusion of the proximal subclavian artery. It presents with transient ischemic episodes of the posterior cerebral circulation.

Case Report: Our patient is a 25-year-old woman presenting with subclavian steal syndrome as the initial presentation of TAK. She was referred to radiology department by the neurologist for a 4-vessel CT angiography for syncopal episodes. Examination findings revealed non-palpable brachial and radial pulses of the both upper limbs with non-recordable blood pressure readings in both arms.

Investigation revealed elevated CRP and normocytic anemia.CT angiography of carotids and aorta showed smooth circumferential intimal thickening of the aorta, non visualization of both subclavian arteries. She is currently being followed by the vascular surgery and rheumatology teams who have recommended medical management till the acute phase is over. The patient is being managed with steroids and methotrexate but her symptoms are not improving.

Conclusion: We emphasize the importance of understanding the varied clinical spectrum of TAK and the need to have a high index of suspicion for TAK in a young female with recurrent syncope and upper extremity intermittent numbness and paresthesia.

Keywords: immune-mediated, Pakistani woman, vasculitis, Takayasu, subclavian steal syndrome