Medical College of Wisconsin, Milwaukee, Wisconsin 52366, USA.
Curr Opin Neurol. 2012 Oct;25(5):573-85. doi: 10.1097/WCO.0b013e3283580432.
Vasculitic neuropathy is a heterogeneous disorder that usually occurs in systemic diseases, but less commonly appears as nonsystemic vasculitic neuropathy (NSVN). This review is intended to highlight recent developments in the field of vasculitic neuropathies.
A Peripheral Nerve Society guideline provides data-driven consensus recommendation on classification of vasculitic neuropathies and diagnosis/treatment of NSVN. NSVN is sometimes accompanied by subclinical inflammation of adjacent skin. Amyotrophic lateral sclerosis with sensory involvement can mimic NSVN. Systemic vasculitides with neuropathy include polyarteritis nodosa, microscopic polyangiitis (MPA), rheumatoid vasculitis, Churg-Strauss syndrome (CSS), and hepatitis C-related mixed cryoglobulinemic vasculitis (MCV). At autopsy, MPA affects limb nerves diffusely, with maximal damage in proximal/middle segments. CSS can be accompanied by antineutrophil cytoplasmic antibodies (ANCAs), but most patients with neuropathy lack ANCAs. Cryoglobulinemic neuropathies are usually caused by vasculitis, irrespective of phenotype. Two randomized trials revealed rituximab to be noninferior to cyclophosphamide for inducing remission in ANCA-associated vasculitis. Many reports also document efficacy of rituximab in MCV.
Consensus guidelines on NSVN should be evaluated prospectively. MPA-associated vasculitic neuropathy results from vasculitic lesions distributed diffusely throughout peripheral extremity nerves. Rituximab is effective for ANCA-associated and cryoglobulinemic vasculitis with neuropathy.
血管炎性神经病是一种异质性疾病，通常发生于系统性疾病，但较少表现为非系统性血管炎性神经病（NSVN）。本文旨在强调血管炎性神经病领域的最新进展。
外周神经学会指南提供了基于数据的共识推荐，用于血管炎性神经病的分类以及 NSVN 的诊断/治疗。NSVN 有时伴有相邻皮肤的亚临床炎症。伴有感觉受累的肌萎缩侧索硬化症可模拟 NSVN。伴有神经病的系统性血管炎包括结节性多动脉炎、显微镜下多血管炎（MPA）、类风湿性血管炎、Churg-Strauss 综合征（CSS）和丙型肝炎相关混合性冷球蛋白血症性血管炎（MCV）。尸检时，MPA 弥漫性影响肢体神经，近端/中段损伤最大。CSS 可伴有抗中性粒细胞胞质抗体（ANCAs），但大多数神经病患者缺乏 ANCAs。冷球蛋白血症性神经病通常由血管炎引起，不论表型如何。两项随机试验显示利妥昔单抗在诱导 ANCA 相关性血管炎缓解方面不劣于环磷酰胺。许多报告还记录了利妥昔单抗在 MCV 中的疗效。
应前瞻性评估 NSVN 的共识指南。MPA 相关性血管炎性神经病是由分布于外周肢体神经的血管炎性病变引起的。利妥昔单抗对伴有神经病的 ANCA 相关性和冷球蛋白血症性血管炎有效。
