巨细胞动脉周围炎
Giant cell arteritis
定义 英文原文（暂无中文）
A rare large vessel vasculitis (LVV) characterized by vasculitis predominantly involving the arteries originating from the aortic arch and the extracranial branches of the carotid arteries. Clinical manifestations are variable, the predominant cranial phenotype is characterized by headache, jaw claudication, scalp tenderness and visual symptoms and the predominant LVV type by constitutional symptoms, polymyalgia rheumatica and occasionally limb ischemia. Overlaps between these two phenotypes are common.
别名
颞动脉炎
基本事实
- 遗传方式
- 多基因/多因素
- 发病年龄
- 成年期
- 患病率
- 1-9 / 100 000（Germany）
相关基因 4
| 基因 | 名称 | 关联类型 |
|---|---|---|
| HLA-B | major histocompatibility complex, class I, B | Major susceptibility factor in |
| HLA-DRB1 | major histocompatibility complex, class II, DR beta 1 | Major susceptibility factor in |
| PTPN22 | protein tyrosine phosphatase non-receptor type 22 | Major susceptibility factor in |
| P4HA2 | prolyl 4-hydroxylase subunit alpha 2 | Major susceptibility factor in |
临床表型 73
极常见 99–80%10
- 厌食症 HP:0002039
- 脑缺血 HP:0002637
- C-反应蛋白水平升高 HP:0011227
- 疲乏 HP:0012378
- 发热 HP:0001945
- 头痛 HP:0002315
- 咀嚼功能受损 HP:0005216
- 关节僵硬 HP:0001387
- 血管炎 HP:0002633
- 体重减轻 HP:0001824
常见 79–30%18
- 全身血压异常 HP:0030972
- 脱发 HP:0001596
- 贫血 HP:0001903
- 关节炎 HP:0001369
- 视力模糊 HP:0000622
- 颈动脉痛 HP:6000944
- 抑郁 HP:0000716
- 循环钙卫蛋白水平升高 HP:6000502
- 循环骨调素水平升高 HP:0033123
- 红细胞沉降速度升高 HP:0003565
- 高纤维蛋白原血症 HP:0011899
- α-球蛋白增加 HP:0005413
- 循环白细胞介素6水平升高 HP:0030783
- 颌跛行 HP:0030164
- 全身乏力不适 HP:0033834
- 眼肌瘫痪 HP:0000597
- 多发性关节炎 HP:0005764
- 视觉障碍 HP:0000505
偶见 29–5%45
- 腹主动脉瘤 HP:0005112
- 腹痛 HP:0002027
- 胸膜异常 HP:0002103
- 血小板异常 HP:0001872
- 颞浅动脉搏动消失 HP:6000786
- 一时性黑蒙 HP:0100576
- 主动脉夹层 HP:0002647
- 心律失常 HP:0011675
- 动脉血栓形成 HP:0004420
- 关节疼痛 HP:0002829
- 共济失调 HP:0001251
- 传导性听力受损 HP:0000405
- 咳嗽 HP:0012735
- 尿崩症 HP:0000873
- 复视 HP:0000651
- 右室双出口伴肺动脉下室间隔缺损无肺动脉狭窄 HP:0011658
- 构音障碍 HP:0001260
- 鼻衄 HP:0000421
- 坏疽 HP:0100758
- 胃肠道梗塞 HP:0005244
- 舌炎 HP:0000206
- 听力受损 HP:0000365
- 血尿 HP:0000790
- 肝功能衰竭 HP:0001399
- 多汗症 HP:0000975
- 纵膈淋巴结病 HP:0100721
- 脑膜炎 HP:0001287
- 肌无力 HP:0001324
- 肌痛 HP:0003326
- 干咳 HP:0031246
- 眼球震颤 HP:0000639
- 视神经萎缩 HP:0000648
- 感觉异常 HP:0003401
- 心包炎 HP:0001701
- 周围神经病 HP:0009830
- 上睑下垂 HP:0000508
- 复发性咽炎 HP:0100776
- 肾功能不全 HP:0000083
- 头皮压痛 HP:0100809
- 皮肤溃疡 HP:0200042
- 心脏性猝死 HP:0001645
- 眩晕 HP:0002321
- 视野缺损 HP:0001123
- 幻视 HP:0002367
- 视力丧失 HP:0000572
近两年的全球研究 1,976L2
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。
- 2026-09综述Undiagnosed Thoracic Aortic Aneurysms: Prevalence, Risk Factors, Screening Modalities
- 2026-08A systematic review of abatacept and belatacept in immune-mediated diseases
- 2026-08ANCA positive Giant Cell Arteritis? A case report of small vessel vasculitis mimicking large vessel vasculitis
- 2026-08Attentive Bedside Cerebrovascular Examination
- 2026-08Serum calprotectin as a biomarker for large vessel vasculitis: a multicenter cross-sectional study
- 2026-08系统综述综述Evolution of the Diagnostic Paradigm for Giant Cell Arteritis: From Histopathology to Multimodal Imaging Integration
- 2026-07Secondary Adrenal Insufficiency and Multisystem Endocrinopathy Following Immune Checkpoint Inhibitor Therapy
- 2026-07综述Large Vessel Vasculitis Revisited: Mechanistic Insights and Advances in Diagnosis and Management
- 2026-07Literature Commentary
- 2026-07Clinical outcomes of upadacitinib continuation vs withdrawal in giant cell arteritis through 2 years
- 2026-07综述Cerebrovascular involvement in Erdheim-Chester disease: a case report and systematic literature review
- 2026-07Thunderclap Headache in a Patient With Giant Cell Arteritis: Consider Aortic Dissection
- 2026-07Severe infections in giant cell arteritis - incidence over time and relation to large vessel involvement and comorbidities, a population-based study
- 2026-07Association Between Baseline Aortic 18F-Fluorodeoxyglucose Uptake and Subsequent Aortic Dilatation in Giant Cell Arteritis: A Four-Year Post Hoc Analysis
- 2026-072025 EULAR recommendations for the management of polymyalgia rheumatica and primary large vessel vasculitis
- 2026-07Utility of the SCVP consensus statement of temporal artery biopsy for arteritis
- 2026-07Correction: Giant Cell Arteritis
- 2026-07Mesenteric Involvement in Large-Vessel Giant Cell Arteritis: Why PET-CT Findings Should Be Paired With Objective Ischemia-Risk and Gastrointestinal Follow-Up Reporting
- 2026-07[Rheumatological care of older people : Annual report 2026 from the National Database of the regional Collaborative Arthritis Centers]
- 2026-07Time to diagnosis in large vessel vasculitis: insights from a retrospective study at a tertiary rheumatology centre
境外已获批用于本病的药物 3L2
欧盟 2 项、美国 1 项。同一药物在两地各批一次的，会分别列出。
「境外已获批」不等于「在中国能用」。中间隔着进口注册、临床急需境外新药通道、海南博鳌乐城国际医疗旅游先行区等几条路径，各有各的条件与费用。这一节能确定地告诉你的只有一件事：这个病在世界范围内已经有获得批准的药物，它叫什么名字。拿这个名字去问主治医生，是下一步最省力的做法。
药名一律保留英文原文，不作翻译——中国的药品通用名与英文名的音译经常不一致，译错会让人去找一个不存在的药。
- RoActemra欧盟2009-01-16tocilizumab官方记录
- Rinvoq欧盟2019-12-16upadacitinib官方记录
- Rinvoq美国2025-04-28upadacitinib官方记录
已获孤儿药资格、尚未获批的在研药物（1 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。
- Abatacept美国2017-02-22Treatment of giant cell arteritis官方记录
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 4L2
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 2
- 招募中NCT06172361Induction and Tapering Therapy With Tofacitinib and Glucocorticoid in Patients With Polymyalgia Rheumatica中国研究中心 1 个：Hangzhou
- 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
其他状态的试验（2 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NCT04799262Tofacitinib as a GC Sparing Agent for Polymyalgia Rheumatica中国研究中心 1 个：Shanghai
中国境外的在招试验 55L2
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。
法国14美国10加拿大7丹麦6德国6英国4瑞士3瑞典1意大利1Armenia1土耳其1
CT.gov 报告命中 55 项，此处取回并展示最近的 15 项。
- 尚未开始招募NCT07269938Mapping B-cell Biology Across the Cardiovascular Territories of Giant Cell Arteritis: Towards a New Therapeutic Approach (RituxiMAP GCA)英国
- 尚未开始招募NCT07737470Inflammatory Disease Biobank for Immunophenotyping and Cardiovascular Research
- 尚未开始招募NCT07749547Using Personalized Rehabilitation to Reduce Disease and Increase Activity of Patients With Polymyalgia Rheumatica
- 尚未开始招募NCT07748195DNA Methylation in PMR
- 尚未开始招募NCT07132164Justification of the Initial Diagnosis and Evaluation of the Overall Evolution of a Cohort of Recent Polymyalgia Rheumatica (JADORE)法国
- 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NCT06833411Treatment for Giant Cell Arteritis With Tocilizumab and 8 as Compared to 26 Weeks of Prednisone瑞士
- 尚未开始招募NCT07720804Kinetics of C-Reactive Protein in Giant Cell Arteritis Following Glucocorticoid Initiation: A Pilot Study (GIDEON)
- 尚未开始招募NCT06957002Bosentan in the Treatment of Giant Cell Arteritis法国
- 招募中NCT07286214Sarilumab Efficacy and Safety in Adults With Early Polymyalgia Rheumatica加拿大、美国
- 招募中NCT07552636Disease Activity Monitoring in Patients With Giant Cell Arteritis Study丹麦
- 尚未开始招募NCT07508514Early Temporal Dynamics of Optic Nerve Sheath Diameter After Therapy in GCA加拿大
- 招募中NCT07246577The VGR GCA Cohort: Ultrasound, Biopsy and Biomarkers - Novel Methods for Diagnosis, Monitoring and Prognosis in Giant Cell Arteritis.瑞典
- 尚未开始招募NCT07354906Upadacitinib in Giant Cell Arteritis (GCA) With Active Large-vessel Involvement.法国
- 招募中NCT07060274Halo Sign Vanishing Time After Steroids Outbreak in GCA Patients法国
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）