IgG4相关疾病

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 1,710L2

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病例报告
  Directional Coronary Atherectomy as a Diagnostic Adjunct in Suspected IgG4-Related Coronary Artery Disease
  JACC. Case reports · DOI · Europe PMC
• 2026-08
  Bilateral IgG4-Related Dacryoadenitis
  Clinical case reports · DOI · Europe PMC
• 2026-08综述
  Rhinosinusitis in Adults With Autoimmune Diseases: A Scoping Review
  International forum of allergy & rhinology · DOI · Europe PMC
• 2026-08病例报告
  Clinical Images: Features of IgG4-related disease in a patient with granulomatosis with polyangiitis
  ACR open rheumatology · DOI · Europe PMC
• 2026-07
  Association of Th2-like inflammation with a Tfh/Tph-germinal center immune programs in submandibular gland lesions of IgG4-related disease
  Rheumatology (Oxford, England) · DOI · Europe PMC
• 2026-07
  Delayed glucocorticoid initiation is associated with lower stent removal rates in IgG4-related retroperitoneal fibrosis: a retrospective study
  Immunological medicine · DOI · Europe PMC
• 2026-07病例报告
  Autoimmune pancreatitis associated with IgG4-related cholangitis: A radiologic-serologic correlation
  Radiology case reports · DOI · Europe PMC
• 2026-07
  A phase 2 trial of rilzabrutinib in IgG4-related disease
  Annals of the rheumatic diseases · DOI · Europe PMC
• 2026-07综述
  [Etiological investigation of inflammatory orbital disease in patients seen in internal medicine]
  La Revue de medecine interne · DOI · Europe PMC
• 2026-07
  Outcome and risk factors for progression in IgG4-related disease under watchful waiting
  Clinical and experimental rheumatology · DOI · Europe PMC
• 2026-07
  IgG4-Related Hepatic Disease Unmasking Hepatic Actinomycosis
  Journal of clinical immunology · DOI · Europe PMC
• 2026-07
  Estimated prevalence and distribution of causes of immune-mediated hypertrophic pachymeningitis in São Paulo, Brazil
  Clinical rheumatology · DOI · Europe PMC
• 2026-07
  IgG4-related disease presenting with a giant coronary artery aneurysm
  Polish archives of internal medicine · DOI · Europe PMC
• 2026-07
  Epitrochlear Kimura Disease With Concurrent Prurigo Nodularis
  The American Journal of dermatopathology · DOI · Europe PMC
• 2026-07
  [This is how I treat IgG4-related disease]
  Zeitschrift fur Rheumatologie · DOI · Europe PMC
• 2026-07
  Association between autoimmune pancreatitis and pancreatic cancer: a case after long-term steroid therapy
  Clinical journal of gastroenterology · DOI · Europe PMC
• 2026-07
  Clinicopathological Features and Expression of CD30, OCT2, and Cyclin D1 in Rosai-Dorfman Disease: A Retrospective Case Series
  Applied immunohistochemistry & molecular morphology : AIMM · DOI · Europe PMC
• 2026-07
  Transcriptomic Profiling and Drug Repurposing Identify Fostamatinib as a Candidate Therapeutic Agent for IgG4-Related Sialadenitis
  Modern rheumatology · DOI · Europe PMC
• 2026-07综述
  Comparative Diagnostic Accuracy of Cholangioscopy-Based Modalities for Indeterminate Biliary Strictures: A Systematic Review and Network Meta-Analysis
  Digestive diseases and sciences · DOI · Europe PMC
• 2026-07病例报告
  Large Progressive Right Coronary Artery Aneurysm Secondary to IgG4 Disease
  JACC. Case reports · 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。

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 38L2

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 19

• 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
• 招募中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
• 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

中国境外的在招试验 33L2

CT.gov 报告命中 33 项，此处取回并展示最近的 14 项。

• 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 · 2026/07/31Amgen
• 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
• 可获取（拓展性用药）NCT06590051
  AMG 335 Expanded Access Program for IgG4-Related Disease
  拓展性用药Amgen

外部标识与链接