A 17‐Year‐Old with Exertional Vision Loss

Case Presentation:

A 17‐year‐old Korean female presented to the emergency department with a 6‐month history of intermittent dizziness and bilateral vision loss with activity. She described loss of vision as whiteness for 30 seconds on standing, activity, or exertion. She was evaluated by her PCP and was diagnosed with iron‐deficiency anemia 2 months ago. She had a normal MRI of the brain, transthoracic echocardiogram, and ECG. She was treated with iron, but her symptoms were getting progressively worse. She developed intermittent left‐hand numbness and weight loss of 15 pounds. In the ER, vital signs were normal except for low BP in the upper extremities. Blood pressure in the right upper extremity was 86/61, left upper extremity 78/50. Physical examination was remarkable for bilateral carotid bruits, bilateral infraclavicular bruits, diminished left and right radial and ulnar pulses, and positive Allen's test on left and right hands. Carotid duplex ultrasound showed 80%–99% stenosis of bilateral carotid arteries. Laboratory evaluation revealed hemoglobin 11.2 g/dL, platelet count 470, sedimentation rate 96 mm/hour, C‐reactive protein 21.3 mg/L, anti‐uclear antibodies 1.0 units, and detectability of 3 antimyeloperoxidase and antiproteinase antibodies. Magnetic resonance imaging and angiography (MRI/A) of the head and neck showed complete occlusion of the left subclavian artery from its origin to the origin of the left vertebral artery, 60%–65% stenosis of the brachiocephalic artery to the origin of the right subclavian, 99% stenosis of the proximal right common carotid, and 20%–55% stenosis of the left common carotid artery. The patient was diagnosed with Takayasu's arteritis.

Discussion:

Takayasu's arteritis is a rare disease that primarily affects young females of Asian ethnicity. Symptoms include fevers, night sweats, anorexia, weight loss, jaw claudication, visual disturbances, dizziness, syncope, arm claudication, stroke, and ischemic heart disease. Diagnosis is established by evidence of systemic inflammation and vaso‐occlusive disease in a young female, usually under the age of 40, Recommended imaging is MRI/A, which can be used to monitor progression of the disease. Initial treatment usually consists of glucocorticoids in combination with an antiplatelet agent. Surgical evaluation should be considered in severe occlusive disease as in this case.

Conclusions:

A high degree of clinical suspicion is required for the appropriate diagnostic workup. Hospitalists should be aware of common signs and symptoms, diagnostic tests, and management of this rare disease.

Author Disclosure:

A. Roy, none; W. Bosch, none.