Sanna G, Bertolaccini M L, Mathieu A
Department of Rheumatology, Homerton University Hospital, London E9 6SR, UK.
Lupus. 2003;12(12):935-42. doi: 10.1191/0961203303lu505oa.
Management of central nervous system (CNS) involvement still remains one of the most challenging problems in systemic lupus erythematosus (SLE). The best available evidence for the treatment of CNS lupus is largely based on retrospective series, case reports and expert opinion. Current therapy is empirical and tailored to the individual patient. Symptomatic, immunosuppressive and anticoagulant therapies are the main strategies for the management of CNS lupus. The choice depends on the most probable underlying pathogenic mechanism and the severity of the presenting neuropsychiatric symptoms. Thrombotic and nonthrombotic CNS disease needs to be differentiated and requires different management strategies. However, this is often challenging since many, if not most CNS manifestations, may be due to a combination of different pathogenic mechanisms and multiple CNS events may occur in the individual patient. Patients with mild manifestations may need symptomatic treatment only, whereas more severe acute nonthrombotic CNS manifestations may require pulse intravenous cyclophosphamide. Plasmapheresis may also be added in patients with more severe illness refractory to conventional treatment. Recently, the use of intrathecal methotrexate and dexamethasone has been reported in a small series of patients, with a good outcome in patients with severe CNS manifestations. Anticoagulation is warranted in patients with thrombotic disease, particularly in those with the antiphospholipid syndrome (APS). This article reviews the clinical approach to therapy in patients with CNS lupus.
中枢神经系统（CNS）受累的管理仍然是系统性红斑狼疮（SLE）中最具挑战性的问题之一。治疗中枢神经系统狼疮的最佳现有证据很大程度上基于回顾性系列研究、病例报告和专家意见。目前的治疗是经验性的且针对个体患者量身定制。对症治疗、免疫抑制治疗和抗凝治疗是管理中枢神经系统狼疮的主要策略。选择取决于最可能的潜在致病机制和所呈现的神经精神症状的严重程度。血栓性和非血栓性中枢神经系统疾病需要加以区分，且需要不同的管理策略。然而，这通常具有挑战性，因为即便不是大多数，许多中枢神经系统表现可能是不同致病机制共同作用的结果，而且个体患者可能会发生多种中枢神经系统事件。轻度表现的患者可能仅需对症治疗，而更严重的急性非血栓性中枢神经系统表现可能需要静脉注射环磷酰胺冲击治疗。对于传统治疗难治的更严重疾病患者，也可加用血浆置换。最近，有报道称在一小部分患者中使用鞘内注射甲氨蝶呤和地塞米松，对有严重中枢神经系统表现的患者取得了良好疗效。有血栓性疾病的患者，尤其是抗磷脂综合征（APS）患者，需要进行抗凝治疗。本文综述了中枢神经系统狼疮患者的临床治疗方法。
