慢性炎症性脱髓鞘性多发性神经病

定义 英文原文（暂无中文）

A rare chronic monophasic progressive or relapsing symmetric sensorimotor disorder characterized by progressive muscular weakness with impaired sensation, absent or diminished tendon reflexes, electrodiagnostic features suggestive of demyelination and occasional elevated cerebrospinal fluid (CSF) protein level.

别名

慢性感染性多发性神经根性脱髓鞘性神经病

基本事实

遗传方式

不适用

发病年龄

青少年期、成年期、儿童期、老年期

患病率

1-9 / 100 000（Europe）

临床表型 17

近两年的全球研究 978L2

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
  The Diagnostic Accuracy of the Second Revision of the European Academy of Neurology/Peripheral Nerve Society Guideline for Diagnosing Chronic Inflammatory Demyelinating Polyradiculoneuropathy
  European journal of neurology · DOI · Europe PMC
• 2026-07
  Ultrasound Findings of Chronic Inflammatory Demyelinating Polyneuropathy of the Sciatic Nerve
  Anesthesiology · DOI · Europe PMC
• 2026-07综述
  Coexisting Inflammatory and Hereditary Neuropathies: A Review
  Muscle & nerve · DOI · Europe PMC
• 2026-07
  Vagus Nerve Hypertrophy in Chronic Inflammatory Demyelinating Polyneuropathy Presenting With Vocal Cord Palsy
  Neurology · DOI · Europe PMC
• 2026-07
  Beyond Nerve Size: The Role of Fascicular Architecture in Differentiating Chronic Inflammatory Demyelinating Polyneuropathy (CIDP) From Other Demyelinating Polyneuropathies
  Cureus · DOI · Europe PMC
• 2026-07综述
  Nipocalimab and other FcRn blockers in neuromuscular disorders
  Pharmacology & therapeutics · DOI · Europe PMC
• 2026-07
  Characterizing Combined Central and Peripheral Demyelination-Insights From a Multimodal Comparison With Chronic Inflammatory Demyelinating Polyneuropathy and Multiple Sclerosis
  Muscle & nerve · DOI · Europe PMC
• 2026-06
  Charcot-Marie-Tooth type 4 C misdiagnosed as CIDP: electrodiagnostic pitfalls and genetic confirmation
  Neurogenetics · DOI · Europe PMC
• 2026-06
  Serum inflammatory proteomic signatures define chronic inflammatory demyelinating polyneuropathy and inform on disease activity
  EBioMedicine · DOI · Europe PMC
• 2026-06
  TRPV1 Agonist Capsaicin Enhances Oxidative-Stress Resistance and Regeneration in Dorsal Root Ganglia and Schwann Cells
  Cells · DOI · Europe PMC
• 2026-06
  Chronic inflammatory demyelinating polyneuropathy with painful ophthalmoplegia: a case report
  Canadian journal of ophthalmology. Journal canadien d'ophtalmologie · DOI · Europe PMC
• 2026-06
  Chimeric antigen receptor-based cell therapies for neuroimmune disorders: Therapeutic advances and structured standard treatment models
  Neural regeneration research · DOI · Europe PMC
• 2026-06开放获取
  RNU4ATAC-opathy: Clinical, molecular, and transcriptomic insights from a large cohort
  Genetics in medicine : official journal of the American College of Med · DOI · Europe PMC
• 2026-06开放获取
  Rethinking EDSS-based ambulation assessment in multiple sclerosis using continuous variable monitoring
  Neurological research and practice · DOI · Europe PMC
• 2026-06
  Association between Proteinuria and Risk of Chronic Inflammatory Demyelinating Polyneuropathy/Multifocal Motor Neuropathy: A Nationwide Population-based Cohort Study
  Journal of epidemiology and global health · DOI · Europe PMC
• 2026-06荟萃分析系统综述开放获取
  The neurological adverse events of immune check point inhibitors in the treatment of cancer
  Journal of neurology · DOI · Europe PMC
• 2026-06开放获取
  Methodological Insights from Low-Vacuum SEM for Morphological Analysis of Schwann Cells on Electrospun Scaffolds
  Polymers · DOI · Europe PMC
• 2026-06综述开放获取
  Biological Drivers of IMiD-Induced Peripheral Neuropathy in Multiple Myeloma
  EJHaem · DOI · Europe PMC
• 2026-06综述开放获取
  Transthyretin Amyloidosis—From Peculiar Neuropathy to a Treatable Chronic Multisystemic Disease
  Genes
• 2026-06开放获取
  The Electrophysiology of Disease Remission in Chronic Inflammatory Demyelinating Polyneuropathy
  European journal of neurology · DOI · Europe PMC

境外已获批用于本病的药物 4L2

欧盟 0 项、美国 4 项。同一药物在两地各批一次的，会分别列出。

• Gamunex-C美国2008-09-12
  Immune Globulin (Human)
  官方记录
• Hizentra美国2018-03-15
  Immune Globulin Subcutaneous (Human), 20% Liquid
  官方记录
• HyQvia美国2024-01-12
  Immune Globulin Infusion 10% (Human) with Recombinant Human Hyaluronidase
  官方记录
• Vyvgart Hytrulo美国2024-06-21
  efgartigimod alfa and hyaluronidase-qvfc
  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 19L2

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 13

• 招募中NCT04561557
  Safety and Efficacy of CT103A Cells for Relapsed/Refractory Antibody-associated Inflammatory Diseases of the Nervous System
  早期 I 期 · 干预性 · 2020/09/22Tongji Hospital
  中国研究中心 1 个：Wuhan
• 招募中NCT05327114
  Efficacy and Safety Study of Nipocalimab for Adults With Chronic Inflammatory Demyelinating Polyneuropathy (CIDP)
  II 期、III 期 · 干预性 · 2022/09/23Janssen Research & Development, LLC
  中国研究中心 19 个：Beijing、Changchun、Changsha、Chengdu、Chifeng、Fuzhou 等 12 地
• 招募中NCT06502015
  Biomarkers in Autoimmune Disease of Nervous System
  观察性 · 2024/07/31Tongji Hospital
  中国研究中心 1 个：Wuhan
• 招募中NCT06290141
  A Study to Test the Efficacy and Safety of Riliprubart Against the Usual Treatment of Intravenous Immunoglobulin (IVIg) in People With Chronic Inflammatory Demyelinating Polyneuropathy (CIDP)
  III 期 · 干预性 · 2024/08/21Sanofi
  中国研究中心 17 个：Beijing、Changsha、Chengdu、Fuzhou、Guangzhou、Hangzhou 等 12 地
• 招募中NCT06858579
  A Study to Evaluate the Efficacy and Safety of DNTH103 in Adults With Chronic Inflammatory Demyelinating Polyneuropathy (CAPTIVATE)
  III 期 · 干预性 · 2025/02/10Dianthus Therapeutics
  中国研究中心 14 个：Beijing、Changsha、Chengdu、Chifeng、Fujian、Guangdong 等 14 地
• 尚未开始招募NCT06485232
  Universal CAR-T Cells in Patients with Refractory Autoimmune Diseases of the Nervous System.
  早期 I 期 · 干预性 · 2025/02/28Xuanwu Hospital, Beijing
  中国研究中心 1 个：Beijing
• 仅邀请入组NCT06859099
  Long-term Safety and Efficacy Study of Riliprubart in Participants With CIDP
  III 期 · 干预性 · 2025/04/01Sanofi
  中国研究中心 8 个：Beijing、Chengdu、Fuzhou、Guangzhou、Shanghai、Wuhan
• 招募中NCT07022197
  Safety and Efficacy of BAFF-R CART for Refractory Neuroimmune Diseases
  I 期、II 期 · 干预性 · 2025/04/10Tianjin Medical University General Hospital
  中国研究中心 1 个：Tianjin
• 招募中NCT06939166
  Universal Chimeric Antigen Receptor T-Cell （UCAR T-cell） Therapy Targeting CD19/B Cell Maturation Antigen （CD19/BCMA） in Patients With r/r Neurological Autoimmune Diseases
  早期 I 期 · 干预性 · 2025/06/17Tianjin Huanhu Hospital
  中国研究中心 1 个：Tianjin
• 招募中NCT07091630
  A Study to Assess the Efficacy and Safety of Empasiprubart in Adults With CIDP
  III 期 · 干预性 · 2025/09/16argenx
  中国研究中心 5 个：Beijing、Fuzhou、Guangzhou、Xi'an
• 招募中NCT07337785
  CD19/BCMA-Targeted UCAR-T for Patients With Neurological Autoimmune Diseases
  早期 I 期 · 干预性 · 2025/12/12Tongji Hospital
  中国研究中心 1 个：Wuhan
• 尚未开始招募NCT07333196
  Tongji NADs Cohort
  观察性 · 2026/03/01Tongji Hospital
  中国研究中心 1 个：Wuhan
• 招募中NCT07526493
  Safety and Pharmacodynamics of QH103 Cell Injection in the Treatment of Patients With Relapsed/Refractory Antibody-Mediated Neurological Autoimmune Diseases.
  I 期 · 干预性 · 2026/04/01Tongji Hospital
  中国研究中心 1 个：Wuhan

中国境外的在招试验 49L2

CT.gov 报告命中 49 项，此处取回并展示最近的 15 项。

• 尚未开始招募NCT07719153
  Ultrasound-driven Stratification in CIDP
  观察性 · 2026/09/01Centre Hospitalier Universitaire de Nice
• 尚未开始招募NCT07638566
  A Study to Assess the Correct Dose, Safety and Efficacy of Empasiprubart in Adolescent Participants Aged 12 to Less Than 18 Years With Chronic Inflammatory Demyelinating Polyradiculoneuropathy
  II 期、III 期 · 干预性 · 2026/09argenx
• 招募中NCT06752356
  A Study Investigating Intravenous Human Normal Immune Globulin (IGIV) 10% KIg10 (QIVIGY) in Subjects With Chronic Inflammatory Demyelinating Polyneuropathy (CIDP)
  III 期 · 干预性 · 2026/08Kedrion S.p.A.
  美国
• 尚未开始招募NCT07696377
  An Exploratory Study of Obinutuzumab β in the Treatment of Chronic Inflammatory Demyelinating Polyradiculoneuropathy
  I 期、II 期 · 干预性 · 2026/07/15Zhongming Qiu
• 招募中NCT07304154
  A Study Evaluating the Safety and Efficacy of KITE-363 in Relapsed/Refractory Autoimmune Neurologic Diseases
  I 期 · 干预性 · 2026/04/10Kite, A Gilead Company
  澳大利亚、加拿大、美国
• 招募中NCT07540221
  A Study to Evaluate the Pharmacokinetics and Safety of XEMBIFY Versus Gamunex-C in Participants With Chronic Inflammatory Demyelinating Polyradiculoneuropathy
  III 期 · 干预性 · 2026/04/02Grifols Therapeutics LLC
  美国
• 招募中NCT07478172
  Effects of Whole-body Electrical Muscle Stimulation Exercise on Adults With Neuromuscular Disease
  不适用 · 干预性 · 2026/03/10University of Missouri-Columbia
  美国
• 招募中NCT07273903
  Subcutaneous Immunoglobulin Therapy Effectiveness Monitoring in CIDP Patients Using Smart Devices
  观察性 · 2026/01/15Heinrich-Heine University, Duesseldorf
  德国
• 招募中NCT07154524
  Immunoadsorption for Treatment of Chronic Inflammatory Demyelinating Polyneuropathy (CIDP)
  观察性 · 2025/08/27University of Ulm
  德国
• 招募中NCT06920004
  A Study to Assess Efficacy and Safety of Empasiprubart Versus IVIg in Adults With CIDP
  III 期 · 干预性 · 2025/08/22argenx
  阿根廷、奥地利、哥伦比亚、捷克、爱沙尼亚、法国、德国、希腊 等 28 国
• 招募中NCT07264426
  Real-World Efgartigimod Effectiveness in CIDP: A Prospective Study
  观察性 · 2025/08/11argenx
  德国、美国
• 尚未开始招募NCT07121985
  Multispectral Optoacoustic Imaging for the Detection of Inflammation and Damage of Peripheral Nerves in Guillain-Barré Syndrome and Chronic Inflammatory Demyelinating Polyneuropathy
  观察性 · 2025/08/06University of Erlangen-Nürnberg Medical School
• 尚未开始招募NCT07006597
  Vitaccess Real CIDP Registry
  观察性 · 2025/07/31Vitaccess Ltd
• 招募中NCT06798012
  A Study of TAK-411 in Adults With Chronic Inflammatory Demyelinating Polyradiculoneuropathy (CIDP)
  II 期 · 干预性 · 2025/05/14Takeda
  加拿大、哥伦比亚、美国
• 招募中NCT06680037
  A Study to Assess the Safety and Clinical Activity of Azer-cel in Participants With B-cell Mediated Autoimmune Disorders
  I 期 · 干预性 · 2025/05/06TG Therapeutics, Inc.
  美国

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