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IgG4相关疾病

IgG4-related disease

ORPHA:284264疾病组中国目录 第1批 · 56

别名

免疫球蛋白G4相关硬化性疾病

基本事实

遗传方式
不适用
发病年龄
成年期

近两年的全球研究 1,897L2

2024/10 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。

  • 2026-11开放获取
    PSC-DM: A Validated Clinical Diagnostic Model for Distinguishing Primary From Secondary Sclerosing Cholangitis
    Liver international : official journal of the International Associatio · DOI · Europe PMC
  • 2026-10开放获取
    IgG4-to-IgG Ratio and Orbital Involvement Predict Clinical Response to B Cell Depletion in IgG4-Related Disease
    ACR open rheumatology · DOI · Europe PMC
  • 2026-09开放获取
    A Retrospective Review of 1821 Cases of Eosinophilia in a Single-Center Study in Eastern China
    EJHaem · DOI · Europe PMC
  • 2026-09病例报告开放获取
    When seizure control comes at a cost: valproic acid-induced necrotising pancreatitis in a patient receiving antiepileptic polypharmacy
    BMJ case reports · DOI · Europe PMC
  • 2026-09开放获取
    Eosinophilic Sialodochitis Identified in Duct Remnant After Submandibular Gland Resection
    World journal of otorhinolaryngology - head and neck surgery · DOI · Europe PMC
  • 2026-09
    Delayed and multisystem immune-related adverse events in MSI-high colon cancer treated with nivolumab plus ipilimumab
    Clinical journal of gastroenterology · DOI · Europe PMC
  • 2026-09开放获取
    Rheumatologists' expectations and perceptions of T-cell redirecting therapies: results from a cross-sectional survey
    Rheumatology international · DOI · Europe PMC
  • 2026-09
    Isolated pulmonary IgG4-related disease mimicking infection and malignancy: clinical features and a proposed diagnostic approach
    Clinical rheumatology · DOI · Europe PMC
  • 2026-09病例报告开放获取
    A case report: an unusual case of ulcerative intestinal IgG4-related disease with clinical features overlapping with probable intestinal Behcet's disease
    BMC gastroenterology · DOI · Europe PMC
  • 2026-09开放获取
    SCTS Annual Meeting 2025: Abstracts
    Journal of cardiothoracic surgery · DOI · Europe PMC
  • 2026-09病例报告
    Cutaneous IgG4-Related Disease Accompanied by Granulomatous Vasculitis: A Case Report
    Journal of cutaneous pathology · DOI · Europe PMC
  • 2026-09综述
    Fibroblast Activation Protein-Directed PET: Expanding FAPI Beyond Cancer
    AJR. American journal of roentgenology · DOI · Europe PMC
  • 2026-09
    Orbital granulomatosis with polyangiitis: MRI discriminators from IgG4-related disease and lymphoma
    The British journal of radiology · DOI · Europe PMC
  • 2026-09
    Leukopenia and thrombocytopenia in immunoglobulin G4-related disease: frequency, severity, and the association with splenomegaly
    Immunological medicine · DOI · Europe PMC
  • 2026-09开放获取
    Sjögren's Disease-Associated Parotid MALT Lymphoma: Diagnostic Pitfall of Ultrasound-Guided Core Needle Biopsy
    Diagnostics (Basel, Switzerland) · DOI · Europe PMC
  • 2026-09病例报告开放获取
    Simultaneous Thyroid Eye Disease and IgG4-Related Orbital Disease in a Patient With Diabetes Mellitus: A Case Report
    The American journal of case reports · DOI · Europe PMC
  • 2026-09综述开放获取
    Quantitative total serum IgG4 reporting in human paragonimiasis: a scoping review
    Tropical medicine and health · DOI · Europe PMC
  • 2026-09
    [A case of IgG4-related pia mater inflammation]
    Rinsho shinkeigaku = Clinical neurology · DOI · Europe PMC
  • 2026-09病例报告开放获取
    Diagnostic Reassessment of a Pulmonary Mass Initially Suspected to Represent IgG4-Related Lung Disease: The Complementary Role of Tissue Metagenomic Sequencing
    Diagnostics (Basel, Switzerland) · DOI · Europe PMC
  • 2026-09开放获取
    Proceedings of the 33rd European Paediatric Rheumatology Congress
    Pediatric rheumatology online journal · DOI · Europe PMC

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。

尚未获批的在研药物(1 项)

这些药已被欧盟或美国的监管机构认定为罕见病用药(英文 orphan drug designation,中文行业里通称「孤儿药资格」——「孤儿」说的是这类药市场太小、没有厂商愿意认领,不是在说病人)。但这只是一种监管身份:意味着监管机构给予研发上的激励,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。

  • Humanised Fc-engineered monoclonal antibody against CD19欧盟2018-01-17
    Treatment of IgG4-related disease
    官方记录

数据来自欧洲药品管理局(EMA)的药品与罕见病用药资格公开导出表,以及美国 FDA 的罕见病用药资格数据库(Orphan Drug Product Designation Database)。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 41L2

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 20

  • 招募中NCT01670695
    A Prospective Cohort Study of IgG4RD in China
    观察性 · 2012/01Peking Union Medical College Hospital
    中国研究中心 1 个:Beijing
  • 招募中NCT03023371
    National Registry of IgG4-RD in China
    观察性 · 2016/12Peking Union Medical College Hospital
    中国研究中心 1 个:Beijing
  • 招募中NCT05789030
    Treatment Strategies for IgG4-RD Patients With Superficial Organ Involvement
    不适用 · 干预性 · 2022/07/01Peking Union Medical College Hospital
    中国研究中心 1 个:Beijing
  • 招募中NCT06663618
    Short-term Glucocorticoid Combined with MMF for IgG4-RD
    不适用 · 干预性 · 2023/09/01Wen Zhang
    中国研究中心 1 个:Beijing
  • 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NCT06497387
    Safety and Efficacy of PRG-1801 for Refractory Lupus Nephritis and IgG4-Related Disease
    早期 I 期 · 干预性 · 2024/07Tongji Hospital
    中国研究中心 1 个:Wuhan
  • 招募中NCT06497361
    Safety and Efficacy of PRG-2311 for Refractory Lupus Nephritis and IgG4-Related Disease
    早期 I 期 · 干预性 · 2024/07Tongji Hospital
    中国研究中心 1 个:Wuhan
  • 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
  • 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NCT07104058
    A Study on the Effect of MRD Detected by FAPI PET / CT on the Recurrence of IgG4-RD
    不适用 · 干预性 · 2025/05/10Luo Yaping
    中国研究中心 1 个:Beijing
  • 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NCT07068165
    A Randomized Controlled Study of the Efficacy and Safety of Lenalidomide in the Treatment of Active IgG4-related Disease
    II 期、III 期 · 干预性 · 2025/07/07Peking Union Medical College Hospital
    中国研究中心 1 个:Beijing
  • 招募中NCT07148791
    Exploratory Study of Anti-BCMA-CD19 CAR-T Cell Therapy in Relapsed or Refractory IgG4-Related Disease
    II 期 · 干预性 · 2025/09/05Chinese PLA General Hospital
    中国研究中心 1 个:Beijing
  • 招募中NCT07190196
    A 52-week Study of Rilzabrutinib Efficacy and Safety Compared to Placebo in Adults Diagnosed With IgG4-related Disease
    III 期 · 干预性 · 2025/09/26Sanofi
    中国研究中心 10 个:Beijing、Chengdu、Hangzhou、Jiazhuang、Taiyuan、Wuhan 等 7 地
  • 尚未开始招募NCT07298590
    An Exploratory Clinical Study on the Safety and Efficacy of CD19/BCMA CAR-NK in the Treatment of Relapsed and Refractory IgG4-related Disease
    早期 I 期 · 干预性 · 2025/12/30Changhai Hospital
    中国研究中心 1 个:Shanghai
  • 尚未开始招募NCT07583030
    A Clinical Study to Evaluate LVIVO-TaVec400 for the Treatment of Relapsed/Refractory Autoimmune Diseases
    早期 I 期 · 干预性 · 2026/04/20Union Hospital, Tongji Medical College, Huazhong University of Science and Technology
    中国研究中心 4 个:Beijing、Hefei、Taiyuan、Wuhan
  • 尚未开始招募NCT07593911
    A Study to Evaluate MTM-H-001 Injection in Adult Patients With B-Cell-Related Autoimmune Diseases
    不适用 · 干预性 · 2026/06Beijing GoBroad Hospital
    中国研究中心 1 个:Beijing
  • 尚未开始招募NCT07719543
    Efficacy and Safety of Lenalidomide in Patients With IgG4-Related Disease
    III 期 · 干预性 · 2026/08/01Chinese PLA General Hospital
    中国研究中心 3 个:Beijing、Sanya、Wuhan
  • 尚未开始招募NCT07806045
    Nebulized UC-EVs for Recurrent or Refractory IMIDs With Lung Involvement
    早期 I 期 · 干预性 · 2026/10/14Qiubai Li
    中国研究中心 1 个:Beijing
  • 尚未开始招募NCT07222553
    Study to Evaluate the Pharmacokinetics, Pharmacodynamics, Safety, and Tolerability of Inebilizumab in Pediatric Participants With IgG4-RD
    II 期 · 干预性 · 2026/10/31Amgen
    中国研究中心 2 个:Beijing、Changsha
其他状态的试验(21 项)
  • 状态未知NCT01665196
    18F-FDG PET/CT for IgG4-Related Disease
    早期 I 期 · 干预性 · 2012/09Peking Union Medical College Hospital
    中国研究中心 1 个:Beijing
  • 状态未知NCT01758393
    Glucocorticoids in Patients With IgG4-RD
    II 期、III 期 · 干预性 · 2012/12Peking Union Medical College Hospital
    中国研究中心 1 个:Beijing
  • 状态未知NCT02458196
    Study of Treatment Response on IgG4 Related Disease (IgG4RD)
    II 期 · 干预性 · 2015/04Peking Union Medical College Hospital
    中国研究中心 1 个:Beijing
  • 已完成NCT02703194
    Leflunomide for Maintenance of Remission in IgG4 Related Disease
    IV 期 · 干预性 · 2016/03Chinese PLA General Hospital
    中国研究中心 1 个:Beijing
  • 状态未知NCT02797665
    Treatment of Obstructive Jaundice in Autoimmune Pancreatitis and/or Immunoglobulin G4-related Sclerosing Cholangitis by Corticosteroids
    不适用 · 干预性 · 2016/05Peking Union Medical College Hospital
    中国研究中心 1 个:Beijing
  • 状态未知NCT03368274
    To Evaluate the Clinical Efficacy of Iguratimod in the Treatment of IgG4 Related Disease (IgG4-RD) With Mild Symptom
    IV 期 · 干预性 · 2017/09/01Wen Zhang
    中国研究中心 1 个:Beijing
  • 状态未知NCT03715699
    Leflunomide Treatment for IgG4-RD
    不适用 · 干预性 · 2018/07/01Peking Union Medical College Hospital
    中国研究中心 1 个:Beijing
  • 状态未知NCT04125511
    Characterizing IgG4-RD With 68Ga-FAPI PET/CT
    早期 I 期 · 干预性 · 2019/11/01Peking Union Medical College Hospital
    中国研究中心 1 个:Beijing
  • 状态未知NCT04539197
    Circulating Plasmablast in Diagnosis and Relapse Prediction of IgG4-RD
    观察性 · 2020/06/10Peking Union Medical College Hospital
    中国研究中心 1 个:Beijing
  • 状态未知NCT04124861
    Withdraw Drug in Stable IgG4-Related Disease
    不适用 · 干预性 · 2020/06/23Peking Union Medical College Hospital
    中国研究中心 1 个:Beijing
  • 进行中·不再招募NCT04540497
    A Study of Inebilizumab Efficacy and Safety in IgG4- Related Disease
    III 期 · 干预性 · 2020/12/04Amgen
    中国研究中心 10 个:Beijing、Guandong、Hohhot、Shang’ai、Shenyang、Wuhan
  • 状态未知NCT04660565
    Belimumab Treatment for IgG4-related Disease
    IV 期 · 干预性 · 2021/01Peking Union Medical College Hospital
    中国研究中心 1 个:Beijing
  • 状态未知NCT05789017
    Treatment Strategies for IgG4-RD Patients With Internal Organ Involvement
    不适用 · 干预性 · 2022/07/01Peking Union Medical College Hospital
    中国研究中心 1 个:Beijing
  • 状态未知NCT05463445
    Multicenter Observational Study of PSC and IgG4-SC in China
    观察性 · 2022/07/01RenJi Hospital
    中国研究中心 1 个:Shanghai
  • 状态未知NCT05625581
    Tofatib Treatment for IgG4-related Disease
    观察性 · 2022/11/10Changhai Hospital
    中国研究中心 1 个:Shanghai
  • 进行中·不再招募NCT05662241
    A Phase 3 Study of Obexelimab in Patients With IgG4-Related Disease
    III 期 · 干预性 · 2023/01/09Zenas BioPharma (USA), LLC
    中国研究中心 9 个:Beijing、Chengdu、Shanghai、Shijiazhuang、Taiyuan、Wuhai 等 7 地
  • 状态未知NCT05781516
    Baricitinib Plus Glucocorticoid for Eosinophilia in IgG4-RD
    不适用 · 干预性 · 2023/01/20Wen Zhang
    中国研究中心 1 个:Beijing
  • 已完成NCT07827469
    Tofacitinib in the Treatment of IgG4 Related Diseases
    不适用 · 干预性 · 2023/04/01Peking University People's Hospital
    中国研究中心 1 个:Beijing
  • 状态未知NCT05974683
    Treatment Strategies in IgG4-RD Patients With Re-elevation of Serum IgG4 Level During Maintenance Remission Period
    不适用 · 干预性 · 2023/08/01Peking Union Medical College Hospital
    中国研究中心 1 个:Beijing
  • 状态未知NCT06361745
    Early Clinical Study of UTAA09 Injection in the Treatment of Relapsed/Refractory Autoimmune Diseases
    不适用 · 干预性 · 2024/04/02PersonGen BioTherapeutics (Suzhou) Co., Ltd.
    中国研究中心 1 个:Hefei
  • 进行中·不再招募NCT07339540
    the Safety and Efficacy of Targeted BCMA In Vivo LV Injection for Recurrent or Refractory Autoimmune Diseases
    早期 I 期 · 干预性 · 2025/11/04Tongji Hospital
    中国研究中心 1 个:Wuhan

中国境外的在招试验 33L2

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。

乌克兰1日本1美国1比利时1德国1阿根廷1荷兰1以色列1西班牙1

CT.gov 报告命中 33 项,此处取回并展示最近的 13 项。

  • 尚未开始招募NCT07793903
    BCT301 in Treating Refractory/Recurrent IgG4-related Disease
    不适用 · 干预性 · 2026/10Peking University Third Hospital
  • 尚未开始招募NCT07621939
    A Study of IMM0306 in IgG4-Related Disease
    II 期、III 期 · 干预性 · 2026/07ImmuneOnco Biopharmaceuticals (Shanghai) Inc.
  • 招募中NCT07536841
    Validation of the Odesa Criteria 2026 for Autoimmune Pancreatitis
    观察性 · 2026/05/01Military Medical Clinical Center of the Southern Region, Ukraine
    乌克兰
  • 招募中NCT07061938
    Study to Assess Safety, Efficacy and Persistence of ACE1831, in Subjects With IgG4-Related Disease
    I 期、II 期 · 干预性 · 2026/01/01Acepodia Biotech, Inc.
    日本、美国
  • 招募中NCT07535554
    The Investigators Will Evaluate the Diagnostic Performance of [18F]-AlF-FAPI-74 PET/CT in Inflammatory Disorders and Compare it With the Current Gold Standard for Inflammation, FDG PET/CT, in Three Patient Cohort: Patients Presenting With Fever of Unknown Origin, IgG4-RD and AxSpA.
    II 期、III 期 · 干预性 · 2025/09/17Universitaire Ziekenhuizen KU Leuven
    比利时
  • 招募中NCT06696027
    AYLo - AutoimmunitY and Loss of y
    观察性 · 2024/11/15University of Bonn
    德国
  • 尚未开始招募NCT06655831
    Key Mechanisms of Abnormal T Cell Activation and Differentiation in IgG4-Related Ophthalmic Disease
    观察性 · 2024/11/01Peking University Third Hospital
  • 尚未开始招募NCT06270524
    Study of the C5a/C5aR1 Axis in IgG4-associated Disease: a Potential Therapeutic Target
    不适用 · 干预性 · 2024/09/01Assistance Publique Hopitaux De Marseille
  • 招募中NCT06573723
    Institutional Registry of Rare Diseases
    观察性 · 2024/07/01Hospital Italiano de Buenos Aires
    阿根廷
  • 招募中NCT06285539
    Drug Rediscovery for Rare Immune Mediated Inflammatory Diseases
    II 期 · 干预性 · 2024/03/12UMC Utrecht
    荷兰
  • 尚未开始招募NCT05746689
    Study of Sirolimus in IgG4-related Disease
    不适用 · 干预性 · 2023/03/01Peking University International Hospital
  • 招募中NCT03473912
    Meir Medical Center Rheumatologic Biobank
    观察性 · 2018/04/16Meir Medical Center
    以色列
  • 招募中NCT05681975
    Immunoglobulin G4 and Immunoglobulin E Antibodies in a Population With Adverse Reactions to Foodstuffs-related Symptoms
    观察性 · 2017/10/01Universidad Politecnica de Madrid
    西班牙

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)