734 APHASIC AORTITIS - A CASE REPORT


 A 66yo man, without previous cardiological history, 3-month history of asthenia and left leg claudication, presence of tibial painless nodular lesions, was admitted at our emergency department in the clinical context of an acute aortic syndrome with chest and interscapular pain, fluctuating systemic BP (215/90 ←→ 90/60 mmHg) ​​and 60 mmHg pressure gradient between the arms. EKG documented diffuse negative T waves. Echocardiography revealed normal biventricular morphology and function and normal valve system. TC angiography documented a dilated aortic root (46mm), diffuse thickening of the aortic walls from the valvular plane (8mm), with involvement of the coronary and epiaortic arteries (50% stenosis of left subclavian artery), extended up to the aortic bifurcation to affect the common iliac arteries. Blood chemistry showed high C-reactive protein levels (29 mg/L; n.v. <5 mg/L) and low troponin levels. In the suspicion of an aortic wall hematoma, the patient was rushed to the OR with evidence of a lardaceous-looking aorta and free from wall hematomas. Surgery was stopped. Histological sample of left inguinal artery showed diffuse wall sclerosis and rare intramural lymphocyte infiltration. PET-CT showed an increased metabolism of the aortic walls. On the second postoperative day, the patient developed fluctuating aphasia aggravated by orthostatic position and arterial hypotension. In the suspicion of TIA, CT angiography was performed and showed no acute brain lesions, but a critical stenosis of the right carotid bifurcation, a right vertebral artery occlusion and a left internal carotid subocclusion. DAPT (clopidogrel + aspirin) was initiated. Blood chemistry showed normal WBC count, elevated C-reactive protein (133 mg/L; n.v. < 5 mg/L) and erythrocyte sedimentation rate (90 mm/h; n.v. < 35 mm/h). Blood and urine cultures, bacterial serology (syphilis, tubercolosis), viral serology (HBV, HCV, HIV) and autoantibodies tested (ANA, ENA, ANCA, MPO, PR3) resulted negative. In the context of a great vessel arteritis, IV methylprednisolone was introduced with clinical and biochemical response (C-reactive Protein 10 mg/L). MRI on day 10 demonstrated the presence of two cerebral subacute ischemic lesions with hemorrhagic infarction. Aspirin was discontinued. In suspicion of Takayasu arteritis, methotrexate was initiated with clinical benefit. Takayasu arteritis has an incidence of 2.6/1 million, female:male 8:1 and the age of onset is usually between 15 and 30 years. The cause is unknown. It can manifest as an acute aortic syndrome. It is important to be aware of this disease and to consider it in clinically ambiguous cases like this one.