Neurology, University Hospital of Cologne, Köln, Germany
Institute of Clinical Neurosciences, University of Sydney, Sydney, New South Wales, Australia.
J Neurol Neurosurg Psychiatry. 2019 Sep;90(9):981-987. doi: 10.1136/jnnp-2019-320314. Epub 2019 Apr 16.
Chronic inflammatory demyelinating polyneuropathy (CIDP) is an immune-mediated neuropathy typically characterised by symmetrical involvement, and proximal as well as distal muscle weakness (typical CIDP). However, there are several 'atypical' subtypes, such as multifocal acquired demyelinating sensory and motor neuropathy (Lewis-Sumner syndrome) and 'distal acquired demyelinating symmetric neuropathy', possibly having different immunopathogenesis and treatment responses. In the absence of diagnostic and pathogenetic biomarkers, diagnosis and treatment may be difficult, but recent progress has been made in the application of neuroimaging tools demonstrating nerve hypertrophy and in identifying subgroups of patients who harbour antibodies against nodal proteins such as neurofascin and contactin-1. Despite its relative rarity, CIDP represents a significant economic burden, mostly due to costly treatment with immunoglobulin. Recent studies have demonstrated the efficacy of subcutaneous as well as intravenous immunoglobulin as maintenance therapy, and newer immunomodulating drugs can be used in refractory cases. This review provides an overview focusing on advances over the past several years.
慢性炎症性脱髓鞘性多发性神经病（CIDP）是一种免疫介导的神经病，通常以对称性受累以及近端和远端肌肉无力（典型的 CIDP）为特征。然而，还有几种“非典型”亚型，如多灶获得性脱髓鞘感觉运动神经病（Lewis-Sumner 综合征）和“远端获得性脱髓鞘对称性神经病”，可能具有不同的免疫发病机制和治疗反应。在缺乏诊断和发病生物标志物的情况下，诊断和治疗可能很困难，但神经影像学工具的应用在神经肥大的证明以及识别针对神经束蛋白（如神经束蛋白和接触蛋白-1）的抗体的患者亚组方面取得了一些进展。尽管 CIDP 相对罕见，但它代表了巨大的经济负担，主要是由于免疫球蛋白的昂贵治疗费用。最近的研究表明，皮下和静脉注射免疫球蛋白作为维持治疗是有效的，在难治性病例中可以使用新型免疫调节药物。这篇综述重点介绍了过去几年的进展。
