Clinical Challenge: Aortitis in the Emergency Department

Abstract A 67 yo woman presented to the ED for a sustained episode of palpitations and acute chest pain. Past medical history: suspected psoriatic arthritis previously treated with steroids and hydroxychloroquine; previous suspicion of rheumatic polymyalgia and Horton arteritis, regressed with steroids; gastroesophageal reflux disease; mild hypercholesterolemia and a family history of premature coronary artery disease. In the ED the patient was hemodynamically stable. On physical examination: tachyarrhythmic heart sounds with no murmurs. The peripheral arterial pulses were preserved and symmetrical. The remaining physical examination was unremarkable. ECG: atrial fibrillation at 150 bpm. The D–dimer was increased, normal hs–troponin and normal haemoglobin. On echocardiography: normal biventricular structure and function; normal aortic root with dilatation of proximal ascending aorta (47 mm), without visible intimal flaps. The anterior wall of the proximal ascending aorta was thickened. A moderate circumferential pericardial effusion was present, without signs of hemodynamic impact. On suspicion of an acute aortic syndrome with possible intramural haematoma of an ascending aortic aneurism, an urgent CT scan was ordered. It showed a severe dilatation of ascending aorta (52 mm) involving of the proximal aortic arch. The anterior aortic wall was thickened (5 mm), without clear images of IMH, aortic dissection or penetrating ulcer. The pericardial effusion had a supra–fluid density without blood density. A repeated CT at 6 hours showed no changes. The case was suggestive of an inflammatory aetiology. The patient was admitted to the cardiac intensive care unit. A PET–CT scan showed signs of an inflammatory disease of the great vessels. A serological autoimmunity screening was negative. The patient was diagnosed with inflammatory aortitis (giant cell vs Takayasu arteritis) and started on high dose iv glucocorticoid therapy. Given the high risk of severe aortic complications, adjunctive therapy with tocilizumab was prescribed. The therapy was gradually tapered, with subsequent switch to oral steroids plus tocilizumab. At one and a half month of follow–up, a CT scan was repeated, documenting stable aortic diameters and the disappearance of the abnormal thickness and of the enhancement of the aortic wall. A follow–up PET–CT scan has been scheduled at 6 months time. An elective aortic intervention has been indicated after remission of the inflammatory phase.