周围神经系统的自身免疫。

Angelo Quattrini, Stefano C Previtali, Bernd C Kieseier, Reinhard Kiefer, Giancarlo Comi, Hans-Peter Hartung
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引用次数: 22

摘要

外周神经系统自身免疫性疾病(PNS)包括由异常免疫反应引起的异质组疾病。大多数这些疾病都有严重的发病率,在某些情况下还会导致死亡。即使是那些自我限制的情况也可能表现出严重的残疾,需要住院治疗。尽管其病因仍难以捉摸,但对引起组织功能障碍和结构损伤的病理生理机制的了解正在增加。构成细胞-细胞和细胞-细胞外-基质相互作用分子机制的几种介质的发现揭示了神经免疫相互作用的各个方面。经典的动物模型与新的遗传方法相关联,进一步增加了我们对调节神经系统炎症性疾病的分子途径的理解。这篇综述的目的是描述神经免疫相互作用的主要分子成分的各种类型和功能及其在PNS的主要自身免疫性疾病中的重要性。我们还提供了PNS自身免疫性疾病的临床和病理特征的广泛描述,以及诊断和治疗意义。
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Autoimmunity in the peripheral nervous system.

Autoimmune disorders of the peripheral nervous system (PNS) comprise a heterogeneous group of diseases that result from an aberrant immune response. Most of these disorders present severe morbidity and, in some cases, mortality. Even those conditions that are self-limited may display severe disability and necessitate hospitalization. Although their etiology remains elusive, there is increasing knowledge of the pathophysiological mechanisms causing tissue dysfunction and structural damage. The discovery of several mediators that constitute the molecular mechanisms of cell-cell and cell-extracellular-matrix interactions has revealed insight into various aspects of the neuroimmune interaction. Classic animal models associated with new genetic approaches have further increased our comprehension of the molecular pathways that regulate inflammatory disorders of the nervous system. The aim of this review is to describe various types and functions of the principal molecular components of the neuroimmune interaction and their importance in the principal autoimmune disorders of the PNS. We also provide an extensive description of clinical and pathological features of autoimmune disorders of the PNS, along with diagnostic and therapeutic implications.

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