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抗中性粒细胞胞浆抗体血管炎

Anti-neutrophil cytoplasmic antibody-associated vasculitis

ORPHA:156152疾病组中国目录 第2批 · 7

别名

抗中性粒细胞胞浆抗体相关小血管炎

基本事实

患病率
1-5 / 10 000(Germany)

近两年的全球研究 699L2

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。

  • 2026-08
    Recognition of ear, nose and throat involvement in patients with ANCA-associated vasculitis: from in-depth interviews to insights for the future
    European archives of oto-rhino-laryngology : official journal of the E · DOI · Europe PMC
  • 2026-08综述
    Advances in the treatment of eosinophilic granulomatosis with polyangiitis
    Nature reviews. Rheumatology · DOI · Europe PMC
  • 2026-08
    Trends in Clinical Indications for Plasma Exchange in Adult Patients Between 2008 and 2024 in Bogotá, Colombia
    Journal of clinical apheresis · DOI · Europe PMC
  • 2026-07荟萃分析系统综述综述
    Risk Factors for Pulmonary Involvement in Patients with ANCA-associated Vasculitis: A Systematic Review and Meta-analysis
    Clinical reviews in allergy & immunology · DOI · Europe PMC
  • 2026-07综述
    Research progress on the diagnostic value of ANCA in autoimmune diseases of patients with refractory rhinosinusitis
    Immunobiology · DOI · Europe PMC
  • 2026-07
    Familial associations between ANCA-associated vasculitis and other immune-mediated inflammatory diseases: a case control study
    Rheumatology international · DOI · Europe PMC
  • 2026-07
    Towards a proteomic plasma biomarker panel for diagnosing vasculitis remission
    Nature communications · DOI · Europe PMC
  • 2026-07综述病例报告
    The Effect of Teriparatide in a Brown Tumor Patient With Pathological Hip Fracture: A Case Report and Literature Review
    JBJS case connector · DOI · Europe PMC
  • 2026-07
    Real-world efficacy and safety of avacopan in ANCA-associated vasculitis: a retrospective comparative study
    RMD open · DOI · Europe PMC
  • 2026-07
    Avacopan-Associated Hepatobiliary Adverse Events and Vanishing Bile Duct Syndrome: A PMDA/JADER and FAERS Reporting-Profile Comparison
    Hepatology research : the official journal of the Japan Society of Hep · DOI · Europe PMC
  • 2026-07系统综述
    The pharmacokinetics and its covariates of rituximab in different clinical populations: a systematic review and narrative synthesis
    European journal of clinical pharmacology · DOI · Europe PMC
  • 2026-07
    Genetic associations of eosinophilic granulomatosis with polyangiitis in the Japanese population: exploring similarities and differences with European populations
    RMD open · DOI · Europe PMC
  • 2026-07
    Extracellular vesicles exposing complement split products are associated with kidney involvement in antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV)
    RMD open · DOI · Europe PMC
  • 2026-07病例报告
    [Diffuse bilateral pulmonary infiltrates with multiorgan involvement]
    Zhonghua jie he he hu xi za zhi = Zhonghua jiehe he huxi zazhi = Chine · DOI · Europe PMC
  • 2026-06综述
    Advances in the Diagnosis, Treatment and Prognosis of ANCA-Associated Glomerulonephritis
    Medicina (Kaunas, Lithuania) · DOI · Europe PMC
  • 2026-06病例报告
    Pediatric case of anti-neutrophil cytoplasmic antibody-associated glomerulonephritis with immunoglobulin a deposition diagnosed early through school urinary screening
    CEN case reports · DOI · Europe PMC
  • 2026-06综述
    Intensive Care Management of ANCA-associated Vasculitides: a Narrative Review
    Current rheumatology reports · DOI · Europe PMC
  • 2026-06
    Anti-neutrophil cytoplasmic antibody associated vasculitis: a retrospective epidemiological study in an Aotearoa New Zealand cohort
    The New Zealand medical journal · DOI · Europe PMC
  • 2026-06荟萃分析系统综述综述
    Kidney failure in patients with ANCA-associated vasculitis: A systematic review of outcomes
    Autoimmunity reviews · DOI · Europe PMC
  • 2026-06开放获取
    No benefit of adding glucocorticoids to maintenance treatment in reducing the risk of major relapses in ANCA-associated vasculitis: real life data from a longitudinal cohort study
    RMD open · DOI · Europe PMC

境外已获批用于本病的药物 1L2

欧盟 0 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。

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。

  • Gabexate美国2020-09-18
    Treatment of anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis
    官方记录
  • a humanized IgG4 mAb that binds to hC5aR1 and blocks C5a binding to C5美国2026-02-19
    treatment of anti-neutrophil cytoplasmic antibody-associated vasculitis
    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 63L2

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 42

  • 招募中NCT05315141
    Multicenter Cohort Study of AAV in Hunan of China
    观察性 · 2022/03/16Xiangya Hospital of Central South University
    中国研究中心 1 个:Changsha
  • 招募中NCT05965284
    Efficacy and Safety for Telitacicept in the Remission Maintenance Treatment of ANCA-associated Vasculitis (TTCAZAREM)
    IV 期 · 干预性 · 2023/03/09Chinese SLE Treatment And Research Group
    中国研究中心 1 个:Beijing
  • 仅邀请入组NCT05859997
    Universal CAR-T Cells (BRL-301) in Relapse or Refractory Autoimmune Diseases
    不适用 · 干预性 · 2023/05/17Bioray Laboratories
    中国研究中心 1 个:Shanghai
  • 招募中NCT05962840
    Efficacy and Safety for Rituximab Combined With Telitacicept in the Treatment of ANCA-associated Vasculitis (TTCAAVREM)
    IV 期 · 干预性 · 2023/06/29Chinese SLE Treatment And Research Group
    中国研究中心 1 个:Beijing
  • 招募中NCT06056921
    Safety and Efficacy of CD19 Targeted CAR-T Therapy for Refractory Autoimmune Disease
    I 期 · 干预性 · 2023/08/31Chongqing Precision Biotech Co., Ltd
    中国研究中心 1 个:Taiyuan
  • 招募中NCT05979051
    A Study to Evaluate the Efficacy and Safety of SHR-1703 in Subjects With Eosinophilic Granulomatosis With Polyangiitis (EGPA)
    II 期、III 期 · 干预性 · 2023/11/16Guangdong Hengrui Pharmaceutical Co., Ltd
    中国研究中心 2 个:Beijing、Hangzhou
  • 招募中NCT06277427
    Refractory ANCA Associated Vasculitis and Lupus Nephritis Treated With BCMA-targeting CAR-T Cells
    不适用 · 干预性 · 2024/02/05Lingli Dong
    中国研究中心 1 个:Wuhan
  • 招募中NCT06285279
    The BCMA/CD19 Dual Targeted CAR-T Cell in Participants With Autoimmune Kidney Diseases
    I 期 · 干预性 · 2024/03/04Nanjing University School of Medicine
    中国研究中心 1 个:Nanjing
  • 招募中NCT06379646
    An Clinical Study of YTS109 Cell Injection in Subjects With Recurrent/Refractory Autoimmune Disease
    不适用 · 干预性 · 2024/04/24China Immunotech (Beijing) Biotechnology Co., Ltd.
    中国研究中心 1 个:Shanghai
  • 尚未开始招募NCT06420154
    The Safety and Efficacy of Anti-CD19 CAR-T Cells in Patients With Relapsed/Refractory Autoimmune Diseases
    早期 I 期 · 干预性 · 2024/05/27First Affiliated Hospital of Wenzhou Medical University
    中国研究中心 1 个:Wenzhou
  • 招募中NCT06350110
    Fourth-gen CAR T Cells Targeting BCMA/CD19 for Refractory Systemic Lupus Erythematosus (SLE)
    I 期、II 期 · 干预性 · 2024/07/10Essen Biotech
    中国研究中心 1 个:Beijing
  • 招募中NCT06548607
    Clinical Study on Targeted CD19 or CD19-BCMA CAR-T Therapy for Autoimmune Diseases
    早期 I 期 · 干预性 · 2024/09/14Nanjing Bioheng Biotech Co., Ltd.
    中国研究中心 1 个:Bengbu
  • 招募中NCT06794008
    BCMA-CD19 CAR-T Therapy for Refractory Autoimmune Diseases
    II 期 · 干预性 · 2024/12/26Peking University People's Hospital
    中国研究中心 1 个:Beijing
  • 招募中NCT06775912
    Clinical Study on Targeted CD19/BCMA CAR-T Therapy for Autoimmune Diseases
    早期 I 期 · 干预性 · 2025/01/03Nanjing Bioheng Biotech Co., Ltd.
    中国研究中心 1 个:Lianyungang
  • 招募中NCT06614270
    Anti-CD19 IL-10/IL15 CAR-NK Cells in Refractory/Relapsed Autoimmune Diseases
    不适用 · 干预性 · 2025/01/06Second Affiliated Hospital, Zhejiang University, School of Medicine
    中国研究中心 1 个:Hangzhou
  • 招募中NCT06941129
    CAR T-cell Therapy Targeting CD19 and BCMA in Patients With Relapse/Refractory Autoimmune Diseases
    I 期 · 干预性 · 2025/02/11Institute of Hematology & Blood Diseases Hospital, China
    中国研究中心 1 个:Tianjin
  • 尚未开始招募NCT06978738
    UCAR T-cell Therapy Targeting CD19/ BCMA in Patients With Relapse/ Refractory Autoimmune Diseases
    I 期 · 干预性 · 2025/05Changzhou No.2 People's Hospital
    中国研究中心 1 个:Changzhou
  • 招募中NCT06978647
    A Clinical Study of YTS109 Cell in R/R Autoimmune Diseases
    I 期 · 干预性 · 2025/05/19China Immunotech (Beijing) Biotechnology Co., Ltd.
    中国研究中心 1 个:Beijing
  • 招募中NCT06980597
    A Study of OL-108 in Relapsed/Refractory Autoimmune Diseases
    I 期 · 干预性 · 2025/06Beijing GoBroad Hospital
    中国研究中心 2 个:Beijing
  • 尚未开始招募NCT06986018
    Clinical Study on the Targeted CD19 Universal CAR-T Cell Injection (RD06-04) for the Treatment of IIM and AAV
    早期 I 期 · 干预性 · 2025/06/14Peking University People's Hospital
    中国研究中心 1 个:Beijing
  • 招募中NCT06828042
    Safety and Efficacy of Universal CD19-targeting CAR-γδT Cells in Refractory Autoimmune Diseases
    I 期、II 期 · 干预性 · 2025/07/01Peking University Third Hospital
    中国研究中心 1 个:Beijing
  • 尚未开始招募NCT07033299
    The Safety, Efficacy, and Cellular Metabolic Kinetics of CT1192 in Treating Patients With Anti Neutrophil Cytoplasmic Antibody Associated Vasculitis
    I 期 · 干预性 · 2025/07/01Union Hospital, Tongji Medical College, Huazhong University of Science and Technology
    中国研究中心 1 个:Wuhan
  • 招募中NCT07203404
    A Study of Anti-CD19/BCMA Universal CAR-T Cell Therapy RD06-05 in Patients With Autoimmune Diseases.
    早期 I 期 · 干预性 · 2025/07/24Nanjing Bioheng Biotech Co., Ltd.
    中国研究中心 1 个:Shanghai
  • 招募中NCT07104721
    A Clinical Study of YTS109 Cell for R/R Autoimmune Diseases
    I 期 · 干预性 · 2025/08/04China Immunotech (Beijing) Biotechnology Co., Ltd.
    中国研究中心 1 个:Hefei
  • 招募中NCT07123519
    A Clinical Study of YTS109 Cells for the Treatment of R/R Autoimmune Diseases
    I 期 · 干预性 · 2025/08/13China Immunotech (Beijing) Biotechnology Co., Ltd.
    中国研究中心 1 个:Tianjin

另有 17 项未列出。

其他状态的试验(21 项)
  • 已完成NCT00301652
    MMF Versus CTX in the Induction Treatment of ANCA Associated Vasculitis
    不适用 · 干预性 · 2003/06Nanjing University School of Medicine
    中国研究中心 1 个:Nanjing
  • 状态未知NCT01066208
    American College of Rheumatology/European League Against Rheumatism (ACR/EULAR) Diagnostic and Classification Criteria for Primary Systemic Vasculitis
    观察性 · 2011/01University of Oxford
    中国研究中心 1 个:Beijing
  • 已完成NCT04413149
    Anti-neutrophil Cytoplasmic Antibody in Interstitial Lung Disease.
    观察性 · 2014/04/01Peking Union Medical College Hospital
    中国研究中心 1 个:Beijing
  • 状态未知NCT02126098
    Observation Study of Clinical Manifestation and Outcome in Chinese Patients With Pulmonary Vasculitis
    观察性 · 2014/05Peking Union Medical College Hospital
    中国研究中心 1 个:Beijing
  • 已终止NCT02294344
    The Clinical Efficacy of DFPP in Patients With AAGN
    不适用 · 干预性 · 2014/06Zhi-Hong Liu, M.D.
    中国研究中心 1 个:Nanjing
  • 已完成NCT04973033
    Effect of Tofacitinib in Treating ANCA-associated Vasculitis
    不适用 · 干预性 · 2019/12/01Shanghai Zhongshan Hospital
    中国研究中心 1 个:Shanghai
  • 状态未知NCT04737343
    Comparison of the Efficacy of Leflunomide and Azathioprine for the Maintenance Therapy of ANCA Associated Vasculitis
    不适用 · 干预性 · 2021/06/30Chinese SLE Treatment And Research Group
    中国研究中心 4 个:Beijing、Hefei、Hohhot、Kunming
  • 状态未知NCT04923074
    the Application of Diffusion Tensor Imaging in the Evaluation of Peripheral Neuropathy in ANCA Associated Vasculitis
    观察性 · 2021/07Second Affiliated Hospital, Zhejiang University, School of Medicine
    中国研究中心 1 个:Hangzhou
  • 状态未知NCT04944524
    Comparison of Tofacitinib and Methotrexate in the Maintained Treatment of GPA
    IV 期 · 干预性 · 2021/07/01Shanghai Zhongshan Hospital
    中国研究中心 1 个:Shanghai
  • 已完成NCT05197842
    Efficacy and Safety of Substitution of Glucocorticoid for BDB-001 Injection in Patients With Anti-neutrophil Cytoplasmic Antibody(ANCA)-Associated Vasculitis
    I 期、II 期 · 干预性 · 2022/02/22Staidson (Beijing) Biopharmaceuticals Co., Ltd
    中国研究中心 22 个:Beijing、Changsha、Hangzhou、Hefei、Luoyang、Nanchang 等 16 地
  • 状态未知NCT05353179
    Study on Pharmacokinetics of Meperizumab Injection and NUCALA® in Healthy Male Volunteers
    早期 I 期 · 干预性 · 2022/06Chia Tai Tianqing Pharmaceutical Group Co., Ltd.
    中国研究中心 1 个:Changchun
  • 进行中·不再招募NCT05263934
    Efficacy and Safety of Depemokimab Compared With Mepolizumab in Adults With Relapsing or Refractory Eosinophilic Granulomatosis With Polyangiitis (EGPA)
    III 期 · 干预性 · 2022/07/14GlaxoSmithKline
    中国研究中心 8 个:Beijing、Guangzhou、Hefei、Nanjing、Qingdao、Shanghai 等 8 地
  • 状态未知NCT05969522
    Stratified Therapy on Pediatric AAGN
    IV 期 · 干预性 · 2023/01/01Wang Mo
    中国研究中心 1 个:Chongqing
  • 状态未知NCT06316076
    Safety and Efficacy Study of CD19-CAR-DNT Cells in Autoimmune Diseases
    I 期 · 干预性 · 2023/10/30RenJi Hospital
    中国研究中心 1 个:Shanghai
  • 状态未知NCT06373081
    Anti-CD19-CD3E-CAR-T Cells in Relapsed/Refractory Autoimmune Disease
    不适用 · 干预性 · 2024/04/20Shanghai Changzheng Hospital
    中国研究中心 1 个:Shanghai
  • 已终止NCT06462144
    IMPT-514 in Systemic Lupus Erythematosus, Anca-associated Vasculitis, and Idiopathic Inflammatory Myopathy
    早期 I 期 · 干预性 · 2024/06/13The Affiliated Nanjing Drum Tower Hospital of Nanjing University Medical School
    中国研究中心 3 个:Nanjing、Shanghai、Xuzhou
  • 进行中·不再招募NCT06388941
    Iptacopan in Patients With ANCA Associated Vasculitis
    II 期 · 干预性 · 2024/08/05Novartis Pharmaceuticals
    中国研究中心 3 个:Beijing、Shijiazhuang、Zhengzhou
  • 已撤回NCT06548620
    A Study of RD06-04 in Patients With Active Autoimmune Diseases
    早期 I 期 · 干预性 · 2024/08/31Nanjing Bioheng Biotech Co., Ltd.
    中国研究中心 1 个:Zhengzhou
  • 已暂停NCT06549296
    Clinical Study on Targeted CD19CAR-T Therapy for Autoimmune Diseases
    早期 I 期 · 干预性 · 2024/09/13Nanjing Bioheng Biotech Co., Ltd.
    中国研究中心 1 个:Xuzhou
  • 进行中·不再招募NCT06656962
    The Efficacy and Safety of Treatment with Telitacicept in Antineutrophil Cytoplasmic Antibody-associated Nephritis (AAGN)
    I 期、II 期 · 干预性 · 2024/10/10Renmin Hospital of Wuhan University
    中国研究中心 1 个:Wuhan
  • 已撤回NCT06866080
    A Study of LCAR-AIO in Subjects With Relapsed/Refractory Autoimmune Diseases
    早期 I 期 · 干预性 · 2025/06/17Nanjing Legend Biotech Co.
    中国研究中心 4 个:Hefei、Nanjing、Wenzhou、Xuzhou

中国境外的在招试验 95L2

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。

美国17法国15英国6加拿大6意大利4德国4巴西3荷兰3波兰3土耳其3日本3以色列2瑞士2瑞典2另有 13 个国家/地区

CT.gov 报告命中 95 项,此处取回并展示最近的 15 项。

  • 尚未开始招募NCT07636655
    Immune Monitoring Following B-cell Depletion in ANCA-associated Vasculitis
    观察性 · 2026/09/01Nantes University Hospital
    法国
  • 尚未开始招募NCT07737470
    Inflammatory Disease Biobank for Immunophenotyping and Cardiovascular Research
    观察性 · 2026/09/01Assistance Publique - Hôpitaux de Paris
  • 招募中NCT07556484
    Pharmacokinetics Of Emulsified Avacopan Applied By NG Tube
    I 期 · 干预性 · 2026/08/01Mayo Clinic
    美国
  • 尚未开始招募NCT07746180
    Obinutuzumab Beta Combined With Low-dose Glucocorticoid in New-onset ANCA-Associated Glomerulonephritis
    II 期 · 干预性 · 2026/07/31Chinese PLA General Hospital
  • 招募中NCT07388277
    CC-97540 in Patients With Antineutrophil Cytoplasmic Antibody-associated Vasculitis
    I 期、II 期 · 干预性 · 2026/07/09Marcela V. Maus, M.D.,Ph.D.
    美国
  • 尚未开始招募NCT07490275
    Allogeneic CD19/BCMA-Targeted CAR-γδT Cell Therapy: Safety and Preliminary Pharmacodynamics in Relapsed/Refractory Autoimmune Diseases
    I 期 · 干预性 · 2026/07/01Institute of Hematology & Blood Diseases Hospital, China
  • 尚未开始招募NCT07176546
    TAVNEOS for Otolaryngologic Manifestations of Granulomatosis With Polyangiitis
    II 期、III 期 · 干预性 · 2026/07Robert Spiera, MD
    美国
  • 尚未开始招募NCT06226662
    Study of NM8074 in Patients with Anti-Neutrophil Cytoplasmic Antibody (ANCA)-Associated Vasculitis (AAV)
    II 期 · 干预性 · 2026/06NovelMed Therapeutics
  • 尚未开始招募NCT07444567
    Roll-over Study for Participants Who Have Completed a Previous Clinical Study With Benralizumab (Fasenra) and Benefit From Continued Treatment
    III 期 · 干预性 · 2026/05/25AstraZeneca
  • 招募中NCT07087912
    Safety and Immunogenicity of the Live Attenuated Tetravalent Butantan-Dengue Vaccine in Autoimmune Rheumatic Diseases
    IV 期 · 干预性 · 2026/03/16University of Sao Paulo General Hospital
    巴西
  • 尚未开始招募NCT07373262
    Avacopan Added to Standard-of-care Therapy in ANCA-associated Vasculitis With Severe Kidney Involvement
    III 期 · 干预性 · 2026/03/01University Hospital, Toulouse
    法国
  • 尚未开始招募NCT07451847
    Comparison of a Strategy Based on Clinico-biological Monitoring Versus Pre-emptive Rituximab Treatment in Cases of ANCA Reappearance in Granulomatosis With Polyangiitis and Microscopic Polyangiitis.
    IV 期 · 干预性 · 2026/02Assistance Publique - Hôpitaux de Paris
    法国
  • 招募中NCT07258524
    PET Assessment of Disease Activity and Cardiovascular Disease Risk in ANCA-associated Vasculitis
    观察性 · 2026/01/28University of Edinburgh
    英国
  • 招募中NCT07089121
    Descartes-08 for Children, Adolescents, and Young Adults With Autoimmune Disorders
    I 期、II 期 · 干预性 · 2026/01/14Cartesian Therapeutics
    美国
  • 尚未开始招募NCT07251179
    Characterization of Autoreactive b Lymphocytes in Autoimmune Diseases and Immune Deficiencies
    观察性 · 2026/01/01University Hospital, Strasbourg, France
    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)