Russo Ricardo A G, Katsicas María M
Service of Immunology & Rheumatology, Hospital de Pediatría "Prof. Dr. Juan P. Garrahan", Buenos Aires, Argentina.
Front Pediatr. 2018 Sep 24;6:265. doi: 10.3389/fped.2018.00265. eCollection 2018.
Takayasu arteritis is an idiopathic granulomatous vasculitis of the aorta and its main branches and it constitutes one of the more common vasculitides in children. Inflammation and intimal proliferation lead to wall thickening, stenotic or occlusive lesions, and thrombosis, while destruction of the elastica and muscularis layers originates aneurysms and dissection. Carotid artery tenderness, claudication, ocular disturbances, central nervous system abnormalities, and weakening of pulses are the most frequent clinical features. The diagnosis is usually confirmed by the observation of large vessel wall abnormalities: stenosis, aneurysms, occlusion, and evidence of increased collateral circulation in angiography, MRA or CTA imaging. The purpose of this revision is to address the current knowledge on pathogenesis, investigations, classification, outcome measures and management, and to emphasize the need for timely diagnosis, effective therapeutic intervention, and close monitoring of this severe condition.
高安动脉炎是一种累及主动脉及其主要分支的特发性肉芽肿性血管炎，是儿童中较为常见的血管炎之一。炎症和内膜增生导致管壁增厚、狭窄或闭塞性病变以及血栓形成，而弹性层和肌层的破坏则引发动脉瘤和夹层分离。颈动脉压痛、间歇性跛行、眼部障碍、中枢神经系统异常以及脉搏减弱是最常见的临床特征。诊断通常通过观察大血管壁异常来确定：血管造影、磁共振血管造影（MRA）或计算机断层血管造影（CTA）成像显示的狭窄、动脉瘤、闭塞以及侧支循环增加的证据。本次修订的目的是阐述有关发病机制、检查、分类、结局指标和管理的当前知识，并强调对这种严重疾病进行及时诊断、有效治疗干预和密切监测的必要性。
