Aortoarteritis: Could it be a form of catecholamine-induced vasculitis?
A 21-year-old lady, mother of two children, presented with recurrent episodes of palpitations, excessive sweating and dyspnea for 3 months. She had a family history of pheochromocytoma []. On examination, she had hypertension in the right upper limb (160/100 mmHg) but pulses were feeble in the left upper limb and both lower limbs. Further evaluation revealed elevated plasma free normetanephrine (1405 pg/ml) with normal plasma free metanephrine (23 pg/ml). Computerized tomography (CT) of the abdomen showed bilateral adrenal pheochromocytomas. Magnetic resonance angiogram [] showed dilatation of aortic root and the proximal ascending aorta (largest diameter = 41 mm). Brachiocephalic trunk showed dilatation just before its bifurcation into the right common carotid artery and right subclavian artery, which were normal. The left subclavian artery was occluded from its origin. A short segment, high-grade stenosis of the celiac artery was seen at its origin. The right renal artery also showed a short segment stenosis in its middle part. Her erythrocyte sedimentation rate (56 mm/1st hour) was elevated while antinuclear antibody and rheumatoid factor were negative. After appropriate preoperative management (blood pressure was 130/86 mmHg on prazosin 20 mg/day and atenolol 50 mg/day), she underwent laparoscopic bilateral adrenalectomy. Postoperatively, she had hypocortisolism and was started on prednisolone (1 mg/kg/day) and fludrocortisone (50 μg/day). Currently, she is under regular follow-up by a cardiovascular surgeon and is normotensive on 10 mg/day of amlodipine.