Real-World Multinational Survey of Chronic Inflammatory Demyelinating Polyneuropathy: Disease Characteristics and Therapeutic Landscape

IF 3.2 3区医学 Q1 CLINICAL NEUROLOGY

Journal of the Peripheral Nervous System Pub Date : 2025-08-18 DOI:10.1111/jns.70047

Luis Querol, Simon Rinaldi, Andras Borsi, Giorgio Maria Boggia, Jonathan de Courcy, Yasmin Taylor, Jack Wright, Wisam Karmous, Wim Noel, Charlotte Gary, Gerd Meyer zu Hörste

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Abstract

Background and Aims

Chronic inflammatory demyelinating polyneuropathy (CIDP) is an immune-mediated syndrome characterized by progressive muscle weakness and sensory impairment. Clinical similarities with other neuropathies can cause misdiagnoses and delayed diagnoses. Additionally, a large proportion of patients appropriately treated according to current guidelines still show residual disability. This real-world study aimed to characterize a global cohort of patients with CIDP.

Methods

Data were drawn from the Adelphi CIDP Disease Specific Programme, a cross-sectional survey with retrospective data collection, conducted in China, France, Germany, Italy, Japan, Spain, the United Kingdom, and the United States between September 2022 and April 2023. Neurologists and neuromuscular specialists reported on patient demographic and clinical characteristics at the time of the survey. Patients self-reported treatment satisfaction, disease control, and health-related outcome measures.

Results

Overall, 164 physicians provided data for 1056 patients, with 428 (40.5%) providing self-reported data. Patients were diagnosed with typical CIDP (69.2%) and variant CIDP (30.8%). Overall, initial misdiagnosis occurred in 37.2% of patients, with a median (interquartile range) diagnostic delay of 6.0 (3.0–12.0) months. Maintenance therapy was prescribed for 81.6% of patients, with corticosteroid use ranging from 25.7% in the United States to 80.0% in China. Some patients were dissatisfied by treatment outcomes (11.0%) and symptom control (12.2%). Overall, mean (SD) patient-reported scores were 62.1 (20.4) for I-RODS, 35.0 (11.1) for FACIT fatigue, and 0.662 (0.253) for EQ-5D-5L.

Interpretation

Diagnostic delay and misdiagnoses were common occurrences across typical CIDP and variant CIDP. Despite the use of guideline treatments, there were unmet needs and a continued disease burden for patients.

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慢性炎症性脱髓鞘性多神经病变的世界多国调查：疾病特征和治疗前景

背景和目的慢性炎症性脱髓鞘性多神经病变（CIDP）是一种以进行性肌肉无力和感觉障碍为特征的免疫介导综合征。与其他神经病变的临床相似可导致误诊和延误诊断。此外，根据现行指南进行适当治疗的很大一部分患者仍然表现出残障。这项真实世界的研究旨在描述全球CIDP患者队列的特征。方法数据来自Adelphi CIDP疾病特定计划，这是一项横断面调查，回顾性数据收集，于2022年9月至2023年4月在中国、法国、德国、意大利、日本、西班牙、英国和美国进行。神经学家和神经肌肉专家在调查时报告了患者的人口统计学和临床特征。患者自我报告治疗满意度、疾病控制和健康相关结果测量。结果164名医生为1056名患者提供了数据，其中428名（40.5%）提供了自我报告的数据。诊断为典型CIDP（69.2%）和变异性CIDP（30.8%）。总体而言，37.2%的患者出现了最初的误诊，诊断延迟的中位数（四分位数范围）为6.0（3.0-12.0）个月。81.6%的患者开了维持治疗处方，使用皮质类固醇的比例从美国的25.7%到中国的80.0%不等。部分患者对治疗结果不满意（11.0%），对症状控制不满意（12.2%）。总体而言，患者报告的I-RODS平均（SD）评分为62.1 (20.4)，FACIT疲劳评分为35.0 (11.1)，EQ-5D-5L评分为0.662（0.253）。诊断延迟和误诊在典型CIDP和变异型CIDP中常见。尽管使用了指南治疗方法，但仍存在未满足的需求和患者持续的疾病负担。

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来源期刊

Journal of the Peripheral Nervous System 医学-临床神经学

CiteScore

6.10

自引率

7.90%

发文量

审稿时长

>12 weeks

期刊介绍： The Journal of the Peripheral Nervous System is the official journal of the Peripheral Nerve Society. Founded in 1996, it is the scientific journal of choice for clinicians, clinical scientists and basic neuroscientists interested in all aspects of biology and clinical research of peripheral nervous system disorders. The Journal of the Peripheral Nervous System is a peer-reviewed journal that publishes high quality articles on cell and molecular biology, genomics, neuropathic pain, clinical research, trials, and unique case reports on inherited and acquired peripheral neuropathies. Original articles are organized according to the topic in one of four specific areas: Mechanisms of Disease, Genetics, Clinical Research, and Clinical Trials. The journal also publishes regular review papers on hot topics and Special Issues on basic, clinical, or assembled research in the field of peripheral nervous system disorders. Authors interested in contributing a review-type article or a Special Issue should contact the Editorial Office to discuss the scope of the proposed article with the Editor-in-Chief.