Large vessel vasculitis

Abstract

Takayasu arteritis is a chronic granulomatous disease of the aorta and its major branches that usually affects women during the second and third decades of life, but it has been reported in young children. This review details the clinical, pathological and radiological features, differential diagnoses and management of the condition, focusing chiefly on the disease in children. The recent definition of Takayasu arteritis is discussed. The condition should be considered in patients with unexplained arterial hypertension or unexplained inflammatory syndromes without signs of localization. Since the disease may be life-threatening and progressive, early recognition is necessary to initiate appropriate therapy. Patients with persistent ischaemic symptoms including hypertension might benefit from revascularization procedures.

Fig. 1

Photomicrograph (light microscopy) showing transmural inflammation of the vessel wall

Fig. 2

Digital subtraction angiogram showing a bilateral renal artery stenosis in an 8-year-old girl and (b) right renal artery after dilatation in the same patient. c Juxta-renal aortic involvement with bilateral renal artery stenosis in a 10-year-old girl. d Tight stenosis of the distal descending thoracic aorta in an 8-year-old girl. e Extensive thoraco-abdominal aortic involvement (with permission). f Bilateral subclavian artery occlusion in a 9-year-old boy. Courtesy of Prof. S. Sharma, Department of Cardiac-Radiology, All India Institute of Medical Sciences, New Delhi

Fig. 3

Contrast-enhanced CT scan showing concentric, thickened, aortic walls of the descending thoracic aorta (with permission)