Case Presentation: A previously healthy sixteen-year old Japanese-American female presented to her primary care provider following one week of frontal headache, dizziness, and a feeling of “floating while walking.” Exam revealed upper extremity blood pressure of 148/93 and a loud bruit across her left lower back. A renal ultrasound ruled out renal artery stenosis, but demonstrated weak and delayed blood flow suggesting proximal arterial obstruction.
The patient was empirically prescribed prednisone and referred for CT angiogram (CTA). CTA revealed luminal narrowing of the suprarenal descending aorta to 0.6×0.6cm. She was then referred to the Emergency Department where she presented with systolic blood pressures to 220mmHg with a 40mmHg difference between UE and LE. The patient denied fevers, abdominal pain, vomiting, or vision changes, but endorsed continued headache, dyspnea on exertion, and bilateral lower extremity claudication. On physical exam epigastric and lumbar bruits were appreciated. Radial pulses were 2+ bilaterally, while dorsalis pedis and tibial pulses were barely palpable. She had normal mental status and adequate urine output. Labs revealed a platelet count of 420×109/L and ESR of 24 mm/hr. MRA confirmed the severe narrowing of the suprarenal abdominal aorta without additional sites of vascular disease.
Given the presentation and imaging results, the patient was admitted and diagnosed with Takayasu arteritis. Her inflammation was treated with 3g of Methylprednisolone and Infliximab infusion; acute hypertension was treated with varying combinations of Enalapril, Amlodipine, Hydralazine, and Labetalol. Decreasing her hypertension while preventing under perfusion and lower extremity hypotension was complex, and further complicated by solumedrol-induced hypertension. After seven days she was discharged with strengthened dorsalis pedis pulses, softer lumbar bruit, and symmetric blood pressures.
Discussion: Takayasu arteritis is a rare large vessel vasculitis with an incidence of ~2.6/1,000,000. Patients may present with vague symptoms of headache and malaise, but discovery of elevated and asymmetric blood pressures necessitates prompt diagnosis. Takayasu arteritis requires careful blood pressure control to prevent hypoperfusion distal to sites of vascular obstruction, while hypertensive urgency requires aggressive management to prevent end organ damage and stroke.
Conclusions: Hypertensive urgency in pediatrics is more commonly due to secondary sources than in the adult population. Vascular involvement including autoimmune vasculitides should be suspected with history of claudication and headache, asymmetric pulses and/or blood pressures and bruits on physical exam, and elevated inflammatory markers. Clinical suspicion for Takayasu arteritis should be high in young female patients of Asian descent with these signs and symptoms as prompt management of inflammation improves long-term outcomes.