Division of Neuromuscular Medicine, Department of Neurology, University of Michigan School of Medicine, Ann Arbor, Michigan, USA.
Department of Neurology, Mayo Clinic, Rochester, Minnesota, USA.
Muscle Nerve. 2021 Feb;63(2):157-169. doi: 10.1002/mus.27046. Epub 2020 Sep 11.
Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is characterized by progressive weakness and sensory loss, often affecting patients' ability to walk and perform activities of daily living independently. With the lack of a diagnostic biomarker, the diagnosis relies on clinical suspicion, clinical findings, and the demonstration of demyelinating changes on electrodiagnostic (EDx) testing and nerve pathology. As a result, patients can often be misdiagnosed with CIDP and unnecessarily treated with immunotherapy. Interpreting the EDx testing and cerebrospinal fluid findings in light of the clinical phenotype, recognizing atypical forms of CIDP, and screening for CIDP mimickers are the mainstays of the approach to patients suspected of having CIDP, and are detailed in this review. We also review the currently available treatment options, including intravenous immunoglobulin (IVIg), corticosteroids (CCS), and plasma exchange (PE), and discuss how to approach treatment-refractory cases. Finally, we emphasize the need to adopt objective outcome measures to monitor treatment response.
慢性炎症性脱髓鞘性多发性神经病（CIDP）的特征是进行性无力和感觉丧失，常影响患者独立行走和进行日常生活活动的能力。由于缺乏诊断生物标志物，诊断依赖于临床怀疑、临床发现以及电诊断（EDx）检查和神经病理学显示脱髓鞘改变。因此，患者经常被误诊为 CIDP，并被不必要地用免疫疗法治疗。根据临床表型解释 EDx 检查和脑脊液结果，认识到 CIDP 的不典型形式，并筛查 CIDP 类似物，是怀疑患有 CIDP 的患者的主要方法，这在本综述中进行了详细介绍。我们还回顾了目前可用的治疗选择，包括静脉注射免疫球蛋白（IVIg）、皮质类固醇（CCS）和血浆置换（PE），并讨论了如何处理治疗抵抗的病例。最后，我们强调需要采用客观的疗效评估来监测治疗反应。
