Takayasu大动脉炎
Takayasu arteritis
定义 英文原文（暂无中文）
A rare predominantly large-vessel vasculitis that is characterized by affected aorta and its major branches, but also other large vessels, causing stenosis, occlusion, or aneurysm.
基本事实
- 遗传方式
- 不适用
- 发病年龄
- 青少年期、成年期
- 患病率
- <1 / 1 000 000（Europe）
相关基因 3
| 基因 | 名称 | 关联类型 |
|---|---|---|
| HLA-B | major histocompatibility complex, class I, B | Major susceptibility factor in |
| IL12B | interleukin 12B | Major susceptibility factor in |
| MLX | MAX dimerization protein MLX | Major susceptibility factor in |
临床表型 54
极常见 99–80%9
- 心脏瓣膜形态异常 HP:0001654
- 动脉狭窄 HP:0100545
- 动脉炎 HP:0012089
- 血管扩张 HP:0002617
- 疲乏 HP:0012378
- 发热 HP:0001945
- 高血压危象 HP:0100735
- 血管炎 HP:0002633
- 体重减轻 HP:0001824
常见 79–30%24
- 主动脉瓣形态异常 HP:0001646
- 无脉 HP:0032554
- 贫血 HP:0001903
- 厌食症 HP:0002039
- 关节炎 HP:0001369
- 生主动脉动脉瘤 HP:0004970
- 双臂血压不对称 HP:6000945
- 腿部血压明显高于手臂 HP:0020141
- 颈动脉痛 HP:6000944
- 胸痛 HP:0100749
- 红细胞沉降速度升高 HP:0003565
- 坏疽 HP:0100758
- 头痛 HP:0002315
- 高血压 HP:0000822
- 炎症反应增强 HP:0012649
- 间歇性跛行 HP:0004417
- 偏头痛 HP:0002076
- 肌无力 HP:0001324
- 肌痛 HP:0003326
- 心肌梗死 HP:0001658
- 肺动脉高压 HP:0002092
- 肾动脉狭窄 HP:0001920
- 癫痫发作 HP:0001250
- 皮肤溃疡 HP:0200042
偶见 29–5%21
- 异常言语模式 HP:0002167
- 心内膜形态异常 HP:0004306
- 一时性黑蒙 HP:0100576
- 主动脉瓣反流 HP:0001659
- 关节疼痛 HP:0002829
- 脑缺血 HP:0002637
- 充血性心力衰竭 HP:0001635
- 扩张型心肌病 HP:0001644
- 呼吸困难 HP:0002094
- C-反应蛋白水平升高 HP:0011227
- 结节性红斑 HP:0012219
- 胃肠道梗塞 HP:0005244
- 咯血 HP:0002105
- 全身乏力不适 HP:0033834
- 盗汗 HP:0030166
- 意识下降 HP:0004372
- 视网膜病变 HP:0000488
- 卒中 HP:0001297
- 短暂性脑缺血发作 HP:0002326
- 眩晕 HP:0002321
- 视觉障碍 HP:0000505
近两年的全球研究 1,157L2
2024/08 起在 Europe PMC 检索所得，按发表时间倒序显示最近 20 篇。标题未译成中文——自动翻译需要接入 LLM 服务，尚未引入。
- 2026-08Amygdalar metabolic activity associated with cerebrovascular events and carotid-vertebral artery stenosis in takayasu arteritis
- 2026-08病例报告Prosthetic Aortic Valve Migration Into the Left Ventricular Outflow Tract in Takayasu Arteritis
- 2026-08Autoantibody-Based Models for the Diagnosis and Activity Assessment of Takayasu Arteritis
- 2026-08Serum calprotectin as a biomarker for large vessel vasculitis: a multicenter cross-sectional study
- 2026-07Biological therapy in Takayasu arteritis. Results from the REVAS registry
- 2026-07综述Large Vessel Vasculitis Revisited: Mechanistic Insights and Advances in Diagnosis and Management
- 2026-07病例报告Ischaemic stroke associated with Takayasu arteritis and latent tuberculosis: a diagnostic and therapeutic challenge
- 2026-072025 EULAR recommendations for the management of polymyalgia rheumatica and primary large vessel vasculitis
- 2026-07Beyond Just Inflammation: An Immunophenotyping Compass to Define the Clinical Pathophysiology and Guide the Management of Takayasu Arteritis
- 2026-07Distinct profiles of symptomatic stroke caused by Takayasu arteritis and large-artery atherosclerosis
- 2026-07Time to diagnosis in large vessel vasculitis: insights from a retrospective study at a tertiary rheumatology centre
- 2026-07Infantile Takayasu Arteritis Mimicking Kawasaki Disease in a 3-Month-Old Infant
- 2026-07病例报告Rare Systemic Vasculitides: Polyarteritis Nodosa and Takayasu Arteritis
- 2026-07Diagnostic performance of [18F]FDG PET/CT in large vessel vasculitis management: an umbrella review
- 2026-07Anesthetic management of a high-risk cesarean delivery in a patient with multivessel Takayasu arteritis
- 2026-07Marked Hyperbilirubinemia in a Patient with Intrahepatic Cholestasis of Pregnancy and a UGT1A1 Polymorphism: A Case Report
- 2026-07Serum ferritin as a predictor of major adverse cardiovascular events in Takayasu arteritis: a two-center retrospective cohort study
- 2026-07Efficacy of leflunomide in active Takayasu arteritis: a randomized double-blind placebo-controlled trial subtitle: Takayasu arteritis clinical trial in China (TACTIC)
- 2026-07Distinct clinical outcomes of positron emission tomography/computed tomography-based subtypes in treatment-naïve Takayasu arteritis
- 2026-07Takayasu Arteritis and Giant Cell Arteritis: Results From a Cross-Sectional Study of 96 Italian Patients
境外已获批用于本病的药物 0L2
欧盟与美国均未检索到已获批用于本病的药物。
已获孤儿药资格、尚未获批的在研药物（2 项）
孤儿药资格只是一种监管身份——它意味着监管机构认可这是罕见病用药并给予研发激励，不代表这个药已被证明有效，也不代表将来一定能上市。绝大多数最终不会成药。列在这里是为了看清有哪些方向正在被尝试。
数据来自欧洲药品管理局（EMA）的药品与孤儿药资格公开导出表，以及美国 FDA 孤儿药资格数据库。两边口径不同：欧盟一侧取的是当前状态仍为「已授权」的药品；美国一侧记录的是「曾获批准」这一事实，FDA 的公开表不追踪药物此后是否退市（例如 Relyvrio 于 2024 年撤市，表中仍记为已获批）。请以官方记录页为准。
在中国开展的临床试验 25L2
按病名在 ClinicalTrials.gov 检索、并校验研究中心含中国大陆而来。登记状态不等于现在真的能入组——务必按 NCT 号到原站核实，并与主治医生商量。
登记为可入组 10
- 招募中NCT05388682Treatment of Patients With Takayasu Arteritis Complicated With Pulmonary Artery Involvement中国研究中心 1 个：Beijing
- 招募中NCT03372980A Registry Study on Biomarkers of Takayasu's Arteritis (ARSBTA)中国研究中心 1 个：Beijing
- 招募中NCT07184814Clinical Features and Prognosis of Takayasu's Arteritis With Pulmonary Arteries Involvement中国研究中心 1 个：Beijing
- 招募中NCT04300686A Pilot Study in Severe Patients With Takayasu Arteritis.中国研究中心 1 个：Shanghai
- 招募中NCT05102448Comparison of Tofacitinib and Methotrexate in Takayasu's Arteritis中国研究中心 1 个：Shanghai
- 招募中NCT06178419Remote Ischemic Conditioning for Cerebral Ischemia in Patients With Takayasu Arteritis (TARIC-1)中国研究中心 1 个：Beijing
- 招募中NCT06498089A Randomized, Controlled, Open-label, Multicenter Clinical Trial Comparing the Efficacy and Safety of a Precision Treatment Regimen Based on Clinical-molecular Phenotypes with a Conventional Treatment Regimen in the Treatment of Patients with Active Takayasu's Arteritis中国研究中心 1 个：Shanghai
- 尚未开始招募NCT06766552A Registry Study Assessing PRO, Dosing Patterns, and Safety of Vunakizumab in Patients With General Rheumatic Diseases.中国研究中心 1 个：Hangzhou
- 尚未开始招募NCT07013838The Efficacy and Safety of Deucravacitinib in Takayasu's Arteritis中国研究中心 1 个：Beijing
- 尚未开始招募NCT07451665Efficacy and Safety of Vunakizumab and Ivarmacitinib in the Treatment of Active Takayasu's Arteritis中国研究中心 20 个：Baotou、Beijing、Chengdu、Ch’ang-ch’un、Guanzhou、Hangzhou 等 17 地
其他状态的试验（15 项）
- 状态未知NCT01066208American College of Rheumatology/European League Against Rheumatism (ACR/EULAR) Diagnostic and Classification Criteria for Primary Systemic Vasculitis中国研究中心 1 个：Beijing
- 已完成NCT03189602Pulmonary Artery Involvement in Takayasu's Arteritis中国研究中心 1 个：Beijing
- 状态未知NCT02964364China Takayasu Arteritis Registry (CTA Registry)中国研究中心 1 个：Beijing
- 状态未知NCT03893136The Registry Study of Takayasu Arteritis in East China中国研究中心 1 个：Shanghai
- 已完成NCT02981979Takayasu Arteritis Clinical Trial in China中国研究中心 6 个：Beijing、Nanchang、Shanghai、Shenyang、Ürümqi
- 状态未知NCT03199183Registration Study of Takayasu's Arteritis in China中国研究中心 1 个：Beijing
- 已完成NCT03096275Comparison of Mycophenolate Mofetil and Cyclophosphamide for Active Takayasu's Arteritis中国研究中心 7 个：Beijing、Hohhot、Shijiazhuang、Tianjin、Xian
- 状态未知NCT04366596Drug-eluting Balloon for Treatment of Non-atherosclerotic Renal Artery Stenosis中国研究中心 1 个：Beijing
- 状态未知NCT04137614Drug-coated Balloon for Takayasu Arteritis Associated Renal Artery Stenosis中国研究中心 1 个：Shanghai
- 进行中·不再招募NCT04161898A Study to Evaluate the Efficacy and Safety of Upadacitinib in Participants With Takayasu Arteritis (TAK)中国研究中心 5 个：Beijing、Guangzhou、Shanghai、Shenyang、Shenzhen
- 状态未知NCT04299971Efficiency of Methotrexate and Tofacitinib in Mild and Moderate Patients中国研究中心 1 个：Shanghai
- 状态未知NCT05151848Comparison of Adalimumab and Tofacitinib in the Treatment of Active Takayasu Arteritis中国研究中心 4 个：Beijing
- 状态未知NCT05749666Comparison of Tofacitinib and Prednisolone in the Treatment of Active Takayasu's Arteritis中国研究中心 1 个：Beijing
- 已完成NCT06662721Baricitinib for Refractory Takayasu Arteritis中国研究中心 1 个：Beijing
- 已完成NCT07500467Upadacitinib for Refractory Takayasu Arteritis中国研究中心 1 个：Beijing
中国境外的在招试验 27L2
这些试验在中国没有研究中心，通常无法直接报名——入组一般要求在当地居住并接受随访。列在这里是因为它另有用处：看清楚全世界正在试哪些药、做到了哪一期、由谁在做。把药名和 NCT 编号记下来去问主治医生，或据此进一步查该药是否已在境外获批、是否有拓展性用药（expanded access）通道。
土耳其4美国4英国3加拿大2巴西1法国1Armenia1
CT.gov 报告命中 27 项，此处取回并展示最近的 15 项。
- 尚未开始招募NCT07737470Inflammatory Disease Biobank for Immunophenotyping and Cardiovascular Research
- 尚未开始招募NCT07571577Monitoring Disease Activity in Patients With Takayasu Arteritis With the OMERACT Takayasu Ultrasound Score (OTUS)
- 尚未开始招募NCT07628075Long-Axial Field of View (LAFOV) [18F]FDG PET/CT Imaging in Large Vessel Vasculitis (LVV): Protocol Optimisation Study.英国
- 尚未开始招募NCT07477795Phase II Interventional Study Evaluating Efficacy and Safety of Secukinumab in Active Severe Takayasu Patients
- 招募中NCT07087912Safety and Immunogenicity of the Live Attenuated Tetravalent Butantan-Dengue Vaccine in Autoimmune Rheumatic Diseases巴西
- 尚未开始招募NCT07325357Effect of Pirfenidone on TA Fibrosis
- 招募中NCT07077304EACVI Study on Multimodality Cardiovascular Imaging of Inflammatory Cardiovascular Diseases法国
- 招募中NCT07351422Investigating Management, Perspectives and Attitudes Towards Care in Takayasu Arteritis英国
- 招募中NCT07276087Physical and Psychosocial Parameters in Takayasu Arteritis and Behçet's Disease: A Comparative Study With Healthy Controls土耳其
- 招募中NCT07558382Global Initiative on Takayasu Arteritis (GITA)土耳其
- 招募中NCT07491913Biologic Treatment Withdrawal in Takayasu Arteritis Patients in Sustained Remission土耳其
- 招募中NCT06887062Dapagliflozin and Endothelin Receptor Antagonism in Large Vessel Vasculitis (DERAIL-LVV)英国
- 招募中NCT05904301Armenian NAtionwide REGistry of Systemic Autoimmune and Autoinflammatory DiseasesArmenia
- 招募中NCT02967068VCRC Tissue Repository加拿大、美国
- 招募中NCT02593565Vasculitis Pregnancy Registry美国
外部标识与链接
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本页数据来源
- 疾病定义、同义词、基因、表型、流行病学：Orphanet（CC BY 4.0）
- 中文病名：Orphanet 中文包，冻结于 2020-06-01
- 表型中文标签：HPO 简体中文翻译（CHPO 上游成果）