Magnus T, Melms A, Kötter I, Holle J U
Klinik und Poliklinik für Neurologie, Universitätsklinikum Hamburg-Eppendorf, Hamburg, Deutschland.
Z Rheumatol. 2012 Sep;71(7):551-63. doi: 10.1007/s00393-012-0957-0.
Neurologic complications of vasculitis occur frequently in the form of either peripheral neuropathy or manifestations within the central nervous system (CNS). Primary vasculitis of the CNS is characterized by central nervous system manifestations only with no evidence of systemic disease manifestations. Large vessel vasculitis is particularly associated with central nervous system complications, such as ischemic cerebral infarcts whereas medium size, e.g. polyarteritis nodosa and small vessel vasculitis, e.g. antineutrophil cytoplasmic antibodies (ANCA)-associated vasculitis manifest with peripheral neuropathies and central nervous system complications. The same also holds true for Behçet's disease which affects both large, medium and small sized arteries and veins. Due to the severity of nervous system manifestations a highly potent immunosuppressive therapy (e.g. cyclophosphamide and glucocorticoids) is usually required for remission induction. Virus-associated vasculitis (e.g. hepatitis C-associated cryoglobulinemic vasculitis) should receive antiviral therapy as first line treatment. Chronic damage is frequent in spite of swift initiation of immunosuppressive treatment.
血管炎的神经系统并发症常以外周神经病变或中枢神经系统（CNS）内的表现形式出现。中枢神经系统原发性血管炎仅以中枢神经系统表现为特征，无全身性疾病表现的证据。大血管血管炎尤其与中枢神经系统并发症相关，如缺血性脑梗死，而中等大小血管炎，如结节性多动脉炎，以及小血管血管炎，如抗中性粒细胞胞浆抗体（ANCA）相关血管炎，则表现为外周神经病变和中枢神经系统并发症。白塞病也是如此，它会影响大、中、小动脉和静脉。由于神经系统表现的严重性，通常需要强效免疫抑制治疗（如环磷酰胺和糖皮质激素）来诱导缓解。病毒相关性血管炎（如丙型肝炎相关冷球蛋白血症性血管炎）应接受抗病毒治疗作为一线治疗。尽管迅速开始免疫抑制治疗，但慢性损伤仍很常见。
