[Takayasu arteritis]

Abstract

Takayasu arteritis is an inflammatory arteritis affecting large vessels, predominantly the aorta and its main branches. Thickening of the vessel wall is an early hallmark of the disease and leads to stenosis, thrombosis, and sometimes aneurysm formation. Reported incidence ranges from 1.2 to 2.6/million/year. Women aged 20 to 40 are most likely to suffer from the disease than men. Manifestations are very polymorphous, with presentations ranging from asymptomatic to neurologic catastrophes. Prognosis depends essentially on complications (retinopathy, hypertension, aneurysm, aortic insufficiency) and initial disease aggressivity. Diagnosis is based on imaging methods. Doppler ultrasound, computed tomography, and magnetic resonance imaging are fast and reliable methods for assessing vessel anatomy and luminal status. Positron emission tomography with fluorodeoxyglucose appears to be a highly sensitive and effective method for detecting disease activity, especially since standard inflammatory markers seem ineffective. Until now, corticosteroids have been the treatment of choice. If remission does not occur, methotrexate is added. Percutaneous transluminal angioplasty and sometimes vascular surgery is necessary in cases of critical ischemia or threatening aneurysm. Duration of treatment, choice of second-line treatment, and protocol for tapering medication currently depend more on experience than on evidence-based medicine. Multicenter studies are needed to guide future practice.