OBJECTIVES To evaluate long-term treatment and outcomes of patients with primary central nervous system vasculitis (PCNSV). METHODS In this cohort of 191 consecutive patients with PCNSV seen at Mayo Clinic, Rochester, MN, over 35 years with long-term follow-up we analyzed response to and duration of therapy, frequency of relapses, long-term remission, efficacy of maintenance therapy and initial intravenous glucocorticoid (GC) pulses, survival and degree of disability. We also compared the efficacy of initial IV and oral cyclophosphamide (CYC). RESULTS A favorable initial response was observed in 83% of patients treated with prednisone (PDN) alone, 81% of those treated with PDN and CYC and 95% of those initially treated with PDN and an immunosuppressant other than CYC. One or more relapses were observed in 30% of patients, 35% had discontinued therapy by last follow-up, and 21.5% maintained remission for at least 12 months after discontinuing therapy. Maintenance therapy was prescribed in 19% of all patients and 34% of patients initially treated with CYC and PDN. High disability scores (Rankin 4-6) and deaths were less frequently observed in patients receiving maintenance therapy and more frequently in patients with Aβ-related angiitis. Large vessel involvement and cerebral infarction at diagnosis were associated with a poor treatment response. Aspirin use was positively associated with long-term remission and having gadolinium-enhanced cerebral lesions or meninges was negatively associated. A high disability score at last follow-up and higher mortality rate were associated with increasing age, cerebral infarction and cognitive dysfunction at diagnosis. Lymphocytic vasculitis on biopsy was associated with a more benign course with reduced disability and mortality. Patients initially treated with mycophenolate mofetil had better outcomes compared to those treated with CYC and PDN. No therapeutic advantages were observed in the patients initially treated with intravenous GC pulses. Intravenous and oral CYC were equally effective in inducing the remission. CONCLUSIONS The majority of patients with PCNSV responded to treatment. We found patient subsets with different outcomes. Mycophenolate mofetil may be an effective alternative to CYC.

中文翻译：

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成人原发性中枢神经系统血管炎的长期缓解，复发和维持治疗：单中心的35年经验。

目的评估原发性中枢神经系统血管炎（PCNSV）患者的长期治疗和结局。方法在这35年来长期随访的191例PCNSV患者队列中，他们连续35年来在明尼苏达州罗切斯特市梅奥诊所就诊，我们分析了其对治疗的反应和持续时间，复发频率，长期缓解，维持疗效治疗和初始静脉糖皮质激素（GC）脉冲，生存率和残疾程度。我们还比较了初始静脉注射和口服环磷酰胺（CYC）的疗效。结果在单独使用泼尼松（PDN）治疗的患者中，有83％观察到了良好的初始反应，PDN和CYC治疗的患者中有81％，PDN和非CYC免疫抑制剂的初始治疗中有95％。在30％的患者中观察到一种或多种复发，35％的患者在上次随访后停止了治疗，而21.5％的患者在停止治疗后至少12个月保持缓解。在所有接受CYC和PDN治疗的患者中，有19％和34％的患者开出了维持治疗的处方。在接受维持治疗的患者中，高残疾评分（Rankin 4-6）和死亡的发生频率较低，而与Aβ相关的血管炎患者的死亡频率较高。诊断时大血管受累和脑梗死与不良的治疗反应有关。阿司匹林的使用与长期缓解呈正相关，而g增强的脑损伤或脑膜呈负相关。末次随访时残疾评分高和死亡率高与诊断时年龄增加，脑梗塞和认知功能障碍有关。活检中的淋巴细胞性血管炎与更良性的病程相关，并降低了残疾和死亡率。最初接受霉酚酸酯治疗的患者比接受CYC和PDN治疗的患者有更好的预后。在最初接受静脉内GC脉冲治疗的患者中未观察到治疗优势。静脉和口服CYC在诱导缓解方面同样有效。结论大多数PCNSV患者对治疗有反应。我们发现了具有不同结局的患者亚组。霉酚酸酯可能是CYC的有效替代品。在最初接受静脉内GC脉冲治疗的患者中未观察到治疗优势。静脉和口服CYC在诱导缓解方面同样有效。结论大多数PCNSV患者对治疗有反应。我们发现了具有不同结局的患者亚组。霉酚酸酯可能是CYC的有效替代品。在最初接受静脉内GC脉冲治疗的患者中未观察到治疗优势。静脉和口服CYC在诱导缓解方面同样有效。结论大多数PCNSV患者对治疗有反应。我们发现了具有不同结局的患者亚组。霉酚酸酯可能是CYC的有效替代品。