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[神经精神性系统性红斑狼疮]

[Neuropsychiatric Systemic Lupus Erythematosus].

作者信息

Tanaka Yoshiya

机构信息

The First Department of Internal Medicine, School of Medicine, University of Occupational and Environmental Health.

出版信息

Brain Nerve. 2016 Sep;68(9):1035-1048. doi: 10.11477/mf.1416200550.

Abstract

Central nervous system damage, a major organ manifestation of systemic lupus erythematosus (SLE), causes significant morbidity and mortality. Designating this condition as neuropsychiatric SLE (NPSLE), the American College of Rheumatology defines it as involving the central and peripheral nervous systems and being characterized by various manifestations including stroke, seizures, and psychosis. NPSLE treatment mainly seeks to reduce damage accrual. In patients with NPSLE, the use of high-dose corticosteroids is recommended in combination with immunosuppressants, such as mycophenolate mofetil and intravenous cyclophosphamide pulse therapy. This can be accomplished by controlling the activity of the disease, minimizing the use of corticosteroids, and optimizing the management of comorbidities, including cardiovascular risk factors. An international task force analysis of a treat-to-target strategy for SLE (T2T/SLE) recommended targeting remission, preventing damage, and improving quality of life. Thus, more effective and less toxic treatments, such as those using biologics or kinase inhibitors, are still being developed for the treatment of SLE/NPSLE.

摘要

中枢神经系统损伤是系统性红斑狼疮(SLE)的主要器官表现之一,会导致显著的发病率和死亡率。美国风湿病学会将这种情况定义为神经精神性狼疮(NPSLE),其累及中枢和周围神经系统,表现形式多样,包括中风、癫痫发作和精神病。NPSLE的治疗主要旨在减少损害累积。对于NPSLE患者,建议大剂量使用皮质类固醇,并联合使用免疫抑制剂,如霉酚酸酯和静脉注射环磷酰胺脉冲疗法。这可以通过控制疾病活动、尽量减少皮质类固醇的使用以及优化合并症(包括心血管危险因素)的管理来实现。一个国际特别工作组对SLE的达标治疗策略(T2T/SLE)进行分析后建议实现病情缓解、预防损害并提高生活质量。因此,仍在研发更有效且毒性更小的治疗方法,如使用生物制剂或激酶抑制剂来治疗SLE/NPSLE。

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