Aortoarteritis: Could it be a form of catecholamine-induced vasculitis?
A 17-year-old girl presented with recurrent episodes of palpitation, headache and excessive sweating for 6 months, which were associated with abdominal pain, and dyspnea for the last 1 month. At the time of presentation to hospital, she complained of severe dyspnea, generalized body swelling and right-sided weakness for the last 3 days. Her blood pressure was 280/160 mmHg. Hypertension was initially managed with sodium nitroprusside infusion. Magnetic resonance imaging revealed hypertensive encephalopathy with infarct in the left middle cerebral artery territory. CT from head to pelvis showed bilateral adrenal pheochromocytomas and a paraganglioma at left renal hilum with calcification of abdominal aorta and coronary artery. CT angiogram showed thickening of the aortic wall with characteristic beading of aorta suggestive of aortoarteritis []. It also revealed calcification both common iliac arteries. Her erythrocyte sedimentation rate was normal while antinuclear antibody was negative. Her plasma free normetanephrine (1070 pg/ml) was elevated. After appropriate preoperative preparation (blood pressure was 128/84 mmHg on prazosin 20 mg/day and atenolol 25 mg/day), she underwent right adrenalectomy, left cortical sparing adrenalectomy and excision of paraganglioma at left renal hilum. She had transient postoperative hypertension, which was managed on 5 mg of amlodipoine until 3 months after surgery. Postoperatively, she had normal serum cortisol (16 μg/dL) levels and did not require glucocorticoid or mineralocorticoid replacement. Three months after surgery, she was normotensive without antihypertensive medications. Family screening revealed bilateral pheochromocytomas in one sibling and her father [].