非系统性血管炎性神经病的临床分类及其临床特征、影像学研究进展

Research Advances in Clinical Classification,Characteristics and Imaging of Nonsystemic Vasculitic Neuropathy

非系统性血管炎性神经病(NSVN)是以滋养周围神经的血管受炎性细胞浸润和破坏,继发血管损害和神经缺血为主要表现的一类疾病。NSVN发病机制尚不明确,可能与自身免疫介导的细胞毒性作用相关。NSVN临床以不对称性肢体无力、疼痛及感觉异常为主要表现,以下肢受累为主,病理特征为血管壁炎症细胞浸润伴血管损害。除经典型NSVN外,还有NSVN的亚型和变异型。NSVN实验室指标多正常或轻度异常,典型电生理表现为轴突性损害。此外,磁共振成像、超声等影像学技术在NSVN的诊断、病情评估和监测中也起着越来越重要的作用。

Nonsystemic vasculitic neuropathy( NSVN) is a kind of disease characterized by infiltration and destruction of inflammatory cells in the blood vessels that nourish the peripheral nerves,with secondary vascular damage and nerve ischemia. Its pathogenesis is not clear and may be related to the cytotoxicity mediated by autoimmunity. The main clinical manifestations of NSVN are asymmetric limb weakness,pain and sensory abnormality with lower limbs mostly affected,and the pathological features are inflammatory cell infiltration of vascular wall with vascular damage. Apart from typical NSVN,several variants and subtypes have been distinguished. The laboratory indexes of NSVN are mostly normal or slightly abnormal,and the typical electrophysiological manifestations are axonal damage. Besides,magnetic resonance imaging,ultrasound and other imaging techniques are playing an increasingly important role in the diagnosis,assessment and monitoring of NSVN.