Medicine Department, University of Washington, MS 359860, 325 9th Avenue, Seattle, WA 98104, USA.
Neuroimaging Clin N Am. 2024 Feb;34(1):23-29. doi: 10.1016/j.nic.2023.07.008. Epub 2023 Aug 21.
Primary central nervous system vasculitis (PCNSV) is a vasculitis limited to the brain and spinal cord. Induction therapy often consists of steroids and cyclophosphamide. Maintenance therapy includes a prednisone taper and may be combined with medications such as azathioprine or mycophenolate mofetil. Relapse is common in PCNSV and an increased dose of steroids is often given, sometimes with a change in therapy. Medications such as rituximab and mycophenolate mofetil may be good alternatives in those who do not respond to initial treatment or who have relapse of disease. Mortality rates of 8% to 9% are reported in the literature.
原发性中枢神经系统血管炎（PCNSV）是一种局限于脑和脊髓的血管炎。诱导治疗通常包括类固醇和环磷酰胺。维持治疗包括泼尼松减量，并可能与硫唑嘌呤或霉酚酸酯等药物联合使用。PCNSV 常复发，常给予类固醇剂量增加，有时改变治疗方法。对于初始治疗无反应或疾病复发的患者，利妥昔单抗和霉酚酸酯等药物可能是较好的选择。文献报道的死亡率为 8%至 9%。
