Author/Authors :
Borhani Haghighi, Afshin Shiraz University of Medical Sciences , Mahmoudi Nezhad, Golnoush Sadat Shiraz University of Medical Sciences , Amoozgar, Hamid Shiraz University of Medical Sciences
Abstract :
Takayasu arteritis (TA) is a rare granulomatous pan-arteritis that involves large vessels predominantly the thoracoabdominal aorta and its branches (1). Angiography, particularly subtraction angiography is the procedure of choice for diagnosis of patients with TA and provides an appropriate guide for endovascular procedure (2, 3).
A ten-year-old Afghan girl was referred to the pediatric cardiology ward of Namazee Hospital, Shiraz, Iran due to a history of fever, vascular claudication, and easy fatigability in both lower extremities. In physical examination, pulseless upper extremities and carotid artery bruit were detected. One year prior to this admission, the patient was labeled as a case of Henoch-Schonlein purpura due to her first presentation with rash and fever. In spite of this management, erythrocyte sedimentation rate and C reactive protein levels had not returned to the normal range. Color Doppler sonography of both upper extremities showed the significant thickening of intima and media, diffuse lumen narrowing of both subclavian arteries, subclavian and axillary arteritis. Bringing together all the aforementioned findings, the patient’s impression was changed to the TA; therefore, prednisolone and infliximab was started. The patient was followed at the outpatient clinic. Four months later, she was admitted in the hospital due to decreased level of consciousness following left sided paralysis in both upper and lower extremities and status epilepticus. In her physical exam, motor power of the left upper and lower extremities was 1/5. The brain computerized tomography showed a small size intra-cerebral hemorrhage in the frontal lobe. Brain magnetic resonance angiography showed stenosis in the posterior, middle and anterior cerebral artery. Echocardiography showed fair left ventricular function, hypertrophic left ventricle with dyskinesia of the ventricular septum. She received plasmaphresis, rituximab, prednisolone, anticonvulsants (phenobarbital and phenytoin) and antihypertensive drugs.