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Immune-mediated neuropathies.
Nature Reviews Disease Primers ( IF 76.9 ) Pub Date : 2018-Oct-11 , DOI: 10.1038/s41572-018-0027-2
Bernd C. Kieseier , Emily K. Mathey , Claudia Sommer , Hans-Peter Hartung

Since the discovery of an acute monophasic paralysis, later coined Guillain-Barré syndrome, almost 100 years ago, and the discovery of chronic, steroid-responsive polyneuropathy 50 years ago, the spectrum of immune-mediated polyneuropathies has broadened, with various subtypes continuing to be identified, including chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) and multifocal motor neuropathy (MMN). In general, these disorders are speculated to be caused by autoimmunity to proteins located at the node of Ranvier or components of myelin of peripheral nerves, although disease-associated autoantibodies have not been identified for all disorders. Owing to the numerous subtypes of the immune-mediated neuropathies, making the right diagnosis in daily clinical practice is complicated. Moreover, treating these disorders, particularly their chronic variants, such as CIDP and MMN, poses a challenge. In general, management of these disorders includes immunotherapies, such as corticosteroids, intravenous immunoglobulin or plasma exchange. Improvements in clinical criteria and the emergence of more disease-specific immunotherapies should broaden the therapeutic options for these disabling diseases.

中文翻译:

免疫介导的神经病。

自从大约100年前发现急性单相性麻痹,后来创造了Guillain-Barré综合征以及50年前发现了慢性类固醇反应性多发性神经病以来,免疫介导的多发性神经病的范围已经扩大,各种亚型持续包括慢性炎症性脱髓鞘性多发性神经根神经病(CIDP)和多灶性运动神经病(MMN)。通常,尽管尚未针对所有疾病鉴定出与疾病相关的自身抗体,但推测这些疾病是由于对位于Ranvier淋巴结或周围神经髓鞘的成分的自身免疫引起的。由于免疫介导的神经病的众多亚型,在日常临床实践中进行正确的诊断非常复杂。而且,治疗这些疾病 特别是它们的慢性变异,例如CIDP和MMN,构成了挑战。通常,这些疾病的治疗包括免疫疗法,例如皮质类固醇,静脉内免疫球蛋白或血浆置换。临床标准的改善和更多疾病特异性免疫疗法的出现应拓宽这些致残疾病的治疗选择。
更新日期:2019-01-26
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