目的:总结ANCA相关性血管炎（AAV）患者大动脉受累的临床特点。方法:回顾性分析北京协和医院2013年1月至2018年12月住院的677例AAV患者大动脉受累的临床表现、系统受累、实验室检查、影像学特点、治疗及预后。采用Shapiro-Wilk进行正态性检验。结果:①有大动脉或一级分支受累的AAV患者9例，占同期住院的AAV患者的1.3%，男女比例为2∶1，中位年龄47岁。② 9例患者中肉芽肿性多血管炎（GPA）4例，显微镜下多血管炎（MPA）4例，嗜酸性肉芽肿性多血管炎（EGPA）1例。③大动脉受累平均每例3处，主要集中在7处部位：升主动脉、主动脉弓3例，头臂干（含颈动脉、锁骨下动脉）4例，腹主动脉2例，腹腔干1例，肾动脉2例，累及肱桡动脉1例，髂动脉及下肢动脉2例，累及冠状动脉左主干、前降支、回旋支及右冠状动脉1例。④大动脉病变形式：3例有动脉瘤、动脉扩张和/或夹层，6例有动脉狭窄或闭塞，3例有动脉周围炎。⑤全身疾病活动情况：发现大动脉受累时，原发病多处于活动期，血管炎活动评分平均（19±11）分，五因子评分平均（1±1）分。AAV脏器受累包括肺受累6例，肾脏受累6例，耳鼻喉受累4例，眼部受累1例，神经系统受累3例，消化道受累1例。⑥治疗和预后：全部患者接受激素、免疫抑制剂治疗，1例行升主动脉及主动脉弓置换术。结论:AAV以小血管炎为主，也可有大动脉受累，多见于活动期患者，受累部位以升主动脉、主动脉弓、头臂干等为主，可表现为动脉瘤、动脉夹层、动脉狭窄、动脉周围炎等，需积极治疗原发病，必要时在原发病稳定前提下手术处理受累大动脉。“,”Objective:To summarize the clinical characteristics of aortic involvement in patients with anti-neutrophil cytoplasmic antibodies-associated vasculitis (AAV).Methods:The clinical manifestations, systemic involvement, laboratory tests, imaging characteristics and treatment of aortic involvement in AAV patients admitted to Peking Union Medical College Hospital from January 2013 to December 2018 were retrospectively analyzed. The measurement data was tested by Shapiro-Wilk for normality.Results:Nine patients (1.3% of all AAV patients) were enrolled into our study. The ratio of male to female was 2∶1 and the median age was 47 years old. Of the 9 patients, 4 were granulomatous polyangiitis (GPA), 4 were nicroscopic polyangiitis (MPA) and 1 was eosinophilic granulomatosis with polyangiitis (EGPA). The aorta was involved in an average of 3 locations per case, mainly in 7 locations: 3 ascending aorta and aortic arch, 4 in the head and arm trunk (including carotid and subclavian artery), 2 in the abdominal aorta, and 1 in the abdominal cavity. There were 2 cases of renal artery, 1 case involving brachial radial artery, 2 cases of iliac artery and lower limb artery, and 1 case involving left main coronary artery, anterior descending branch, circumflex branch, and right coronary artery. Aortic lesions: 3 cases had aneurysms, arterial dilatation and/or dissection, 6 cases had arterial stenosis or occlusion and 3 cases had periarteritis. When major arterial involvement was found, the AAV of the patients were mostly active, with an average of (19±11) for the Birmingham Vasculitis Activity Score (BVAS) vasculitis activity score and (1±1) for the Five Factor Score (FFS) score. 6 cases had lung involvement, 6 cases had kidney involvement, 4 cases had ENT involvement, 1 case had eye involvement, 3 cases had nervous system involvement, and 1 case had gastrointestinal involvement. All patients were treated by steroid and immunosuppressant, while 1 case received the operation of ascending aorta and aortic arch replacement.Conclusion:Although small blood vessel are mainly involved in AAV, it may also have aorta involvement, which is more common in patients who have active disease and need more aggressive treatment. The most commonly affected aorta are ascending aorta, aortic arch, and head and arm trunk, which could be manifested as aneurysms, dissections, and arterial stenosis periarteritis. If necessary, surgicall procedure of the affected aorta could be considered when the situation of AAV is stable enough.