Case Presentation: A 55-year-old man with no known past medical history presented with left facial droop, left hemiplegia, and dysarthria for over 48 hours. CT of the head showed a right middle cerebral arterial (MCA) ischemic infarct. Patient was not a candidate for thrombolysis or thrombectomy and was treated with dual antiplatelet therapy. During admission, the patient had repeated episodes of orthostatic hypotension refractory to fluids and midodrine. Adrenal insufficiency was ruled out with a cosyntropin stimulation test. The patient was noted to have a difference of 30 mmHg in systolic blood pressure between his upper and lower extremities. Due to concern for bilateral arterial stenosis, whole body CT angiography was obtained. It demonstrated severe stenosis of the great vessels originating from the aortic arch including the right brachiocephalic artery. The imaging confirmed diagnosis of Takayasu arteritis, which was also determined to be the cause of his stroke. Inflammatory markers were negative at that time. Patient was started on monthly infusions of tocilizumab, a monoclonal antibody targeting interleukin-6 receptors. Repeat imaging showed no progression of disease.
Discussion: Takayasu arteritis (TA) is a form of granulomatous large-vessel vasculitis affecting the aorta and its large branches (1). The disease typically manifests in young women; its mechanism and cause are unknown to this day (3). Important clinical findings are ischemic symptoms of affected organs secondary to arterial stenosis, thrombus formation, and, in rare cases, dissection or ruptured aneurysms (3,4). Our patient’s age and gender are atypical for TA. Additionally, stroke as initial presentation of vasculitis is not common. Studies show a prevalence of stroke in only 6-13% in TA patients. In around half of these cases, a stroke led to initial diagnosis of TA (2,3). The MCA was the most frequently involved artery (3). Interestingly, studies report the absence of systemic inflammatory markers in TA patients presenting with stroke, as seen in this patient (2). The clinical presentation of TA is diverse, and thus a high index of suspicion is needed to avoid misdiagnosis (1). Atypically in this case, there was no asymmetric blood pressure between the upper extremities. Instead blood pressure was disconcordant at the brachial and femoral sites. Blood pressure should be measured in all limbs when there is suspicion for TA. Exam findings suggesting vascular disease in patients with minimal risk factors should raise suspicion for vasculitis, and blood pressure should be measured in all limbs when evaluating for TA.
Conclusions: TA has a highly variable presentation and may initially manifest as stroke. The diagnosis should be considered in patients with apparent vascular disease, especially those with minimal vascular risk factors. Differential blood pressure readings between the upper and lower extremities may provide an important clue to a vascular abnormality.