目的：探讨多发性大动脉炎的全身表现及眼部临床表现，提高眼科医生对大动脉炎的诊治能力。方法：采用回顾性病例系列研究。收集2018年3月至2022年5月山东第一医科大学附属省立医院风湿免疫科确诊后、经眼科会诊或治疗的大动脉炎患者14例，总结患者的全身表现、眼部临床表现及治疗结果。结果：14例多发性大动脉炎患者中，男性1例、女性13例，年龄(35.57±9.77)岁。其中，Ⅰ型头臂动脉型7例(50%)，Ⅱ型胸腹主动脉型2例(14.3%)，Ⅲ型肺动脉型0例，Ⅳ型广泛型5例(35.7%)。85.7%病例累及头臂动脉干。患者全身表现多样，最常见的是上肢低血压相关表现，4例发生严重的心脑血管并发症(28.6%)。5例(35.6%)患者以眼部症状为首发表现，3眼曾误诊为“白内障”(21.4%)。患者最佳矫正视力为无光感~1.0，其中，21眼最佳矫正视力为1.0(占75%)，4眼最佳矫正视力<0.1(占14.3%)。眼部检查正常者4眼(14.3%)，其余24眼(85.7%)均存在眼部异常。晶体异常、结膜巩膜血管扩张充血及瞳孔散大是最常见的眼前节表现。眼底表现以低灌注性视网膜病变为主(20眼，71.4%)，高血压视网膜病变4眼(14.3%)。与Ⅱ型患者比较，Ⅰ型患者更容易发生低灌注性视网膜病变。缺血导致的异常动静脉交通及视盘旁花冠状血管是最典型的眼底表现，严重者导致新生血管形成、增殖性玻璃体视网膜病变和视网膜脱离。所有患者经糖皮质激素和免疫抑制剂治疗，眼部行视网膜光凝及抗血管内皮生长因子(anti-vascular endothelial growth factor,anti-VEGF)药物治疗后，3眼视网膜病变仍有进展，其余患者病情稳定。结论：多发性大动脉炎是一种临床相对比较少见的慢性血管炎性阻塞性病变，年轻女性多发。本组病例以累及头臂动脉干为主，眼部受累概率高，缺血性眼前节表现是较容易被识别的体征，低灌注性视网膜病变是最典型的眼底表现，并可能引发严重的眼部并发症。眼科医生应提高对多发性大动脉炎的认识和诊断能力，并重视对大动脉炎患者的详细眼部检查。

Objective: To discuss the systemic and ocular manifestations of patients with multiplex Takayasu arteritis (TA) and improve the ability of ophthalmologists to diagnose and treat TA. Methods: Retrospective case series study was used. From March 2018 to May 2022, a total of 14 TA patients were admitted to the Department of Rheumatology and Ophthalmology in Shandong Provincial Hospital Affiliated to Shandong First Medical University. They were diagnosed by ophthalmology consultation and treated in the Department of Rheumatology and Immunology. Their systemic manifestations, ocular clinical manifestations, and treatment outcomes were summarized. Results: There were 13 female patients and 1 male patient in our TA cohot. Mean age was (35.57±9.77) years. The most common classification of TA was 7 cases of type I (50%). Others were type II (2 cases of Abdominal aorta and/or renal arteries involved, 14.3%) and 5 cases of type IV (combination of type I and type II, 35.7%), 0 cases of type III. The most common location of involvement was brachiocephalic arterial trunk (85.7%). TA patients had a variety of systemic manifestations, the most common was upper extremity hypotension-related manifestations. Severe cardiovascular and cerebrovascular complications occurred in 4 patients (28.6%). Ocular abnormal was the initial manifestation in 5 patients (35.6%), and 3 eyes were misdiagnosed as cataract (21.4%). Best corrected visual acuity (BCVA) of the TA patients was -1.0 with no light perception, among with 21 eyes had 1.0 (75%) and 4 eyes had a BCVA less than 0.1 (14.3%). Only 4 eyes were totally normal (14.3%) and the remaining 24 eyes (85.7%) had ocular abnormalities. Lens opacity, chronic scleral hyperemia and dilated pupil were the most frequent manifestations in ocular anterior segment. Fundus manifestations were dominated by hypoperfusion retinopathy (20 eyes, 71.4%) and 4 eyes suffered from hypertensive retinopathy (14.3%). Patients with Type I had a higher prevalence for developing hypoperfusion retinopathy than type II patients. Ischemia-induced fiscrete arteriovenous shunt and wreath-like anastomosis around the optic disc were the most typical fundus manifestations. Retinal neovascularization, proliferative vitreoretinopathy and retinal detachment were the most serious retinal complications. Corticosteroids and immunosuppressive agents were administered in all the patients. Most of the patients remained stable, while progression of retinopathy still could be seen in 3 eyes even after pan-retinal photocoagulation and intravitreal injection of anti-VEGF drug treatemnt. Conclusions: TA is a relatively rare chronic vasculitic obstructive lesion that occurs more often in young women. This group of cases mainly involved brachiocephalic artierial trunk. TA has a high possibility of ocular involvement and may lead to various ocular abnormalities. Anterior segment manifestations secondary to ischemia were the relative recognizable signal for diagnosis of TA. Hypoperfusion retinopathy was the most common fundus manifestations, and might cause serious ocular complications. Ophthalmologists should improve awareness and diagnostic capacity for multiple TA and value the detailed eye examination of TA patients.

眼可受全身系统性疾病的累及，通过眼部表现可对心血管系统性疾病、神经系统疾病、自身免疫性疾病、感染性疾病及药物相关眼病等全身疾病进行评估、协助诊断和随访观察。重视全身疾病在眼部的表现，对于眼科及相关专业临床诊疗水平的提升具有重要意义。

Eyes can be affected by systemic diseases. Ocular manifestations can be used to evaluate, help to diagnose and observe systemic diseases including cardiovascular diseases, neurological disorders, autoimmune diseases, infectious diseases and drug-related eye diseases. Paying attention to the manifestations of systemic diseases in the eye is of great significance for the improvement of clinical diagnosis and treatment in ophthalmology and related specialties.