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发布：金银姬 校对：孙琳
摘要
狼疮脑病表现为与系统性红斑狼疮直接相关的神经精神症状。狼疮脑病包括中枢和外周神经系统在内的多种罕见的神经精神症状。由于目前缺乏诊断狼疮脑病的金标准，在诊断狼疮相关的神经精神症状时需严格排除其他可能的潜在病因。在急性期，狼疮脑病患者的治疗和管理与有相同症状的非狼疮患者相同。此后，必须采取基于患者的症状和疾病严重程度的个体化治疗策略。目前仅有极少的有关狼疮脑病的临床试验，大部分数据是从病例分析和病理报告中提取出来的。对于病情严重的患者来说，大剂量糖皮质激素联合环磷酰胺冲击疗法仍然是治疗的关键。若病情未能缓解，则需采用利妥昔单抗、静脉输注免疫球蛋白或血浆置换疗法。当患者表现为轻至中度精神症状或维持治疗有效时可应用硫唑嘌呤或吗替麦考酚酯。当症状考虑是由血栓形成引起时，尤其在抗磷脂抗体阳性或合并抗磷脂综合征时，抗凝治疗和抗血小板治疗必须放在首位。基于对狼疮发病机制的理解，近期应用生物制剂治疗狼疮的试验取得了肯定的效果。本文对目前有关狼疮脑病的流行病学、病理生理学、诊断和治疗管理的文献做了综合的评述。我们将介绍目前狼疮脑病最常用的药物治疗方法以及治疗组可能出现的问题。
附原文：
ABSTRACT: Neuropsychiatric systemic lupus erythematosus (NPSLE) is a generic definition referring to a series of neurological and psychiatric symptoms directly related to systemic lupus erythematosus (SLE). NPSLE includes heterogeneous and rare neuropsychiatric (NP) manifestations involving both the central and peripheral nervous system. Due to the lack of a gold standard, the attribution归属 of NP symptoms to SLE represents a clinical challenge that obligates the strict exclusion of any other potential cause. In the acute setting, management of these patients does not differ from other non-SLE subjects presenting with the same NP manifestation. Afterwards, an individualized therapeutic strategy, depending on the presenting manifestation and severity of symptoms, must be started. Clinical trials in NPSLE are scarce and most of the data are extracted from case series and case reports. High-dose glucocorticoids and intravenous cyclophosphamide remain the cornerstone for patients with severe symptoms that are thought to reflect inflammation or an underlying autoimmune process. Rituximab, intravenous immunoglobulins, or plasmapheresis may be used if response is not achieved. When patients present with mild to moderate NP manifestations, or when maintenance therapy is warranted, azathioprine and mycophenolate may be considered. When symptoms are thought to reflect a thrombotic underlying process, anticoagulation and antiplatelet agents are the mainstay of therapy, especially if antiphospholipid antibodies or antiphospholipid syndrome are present. Recent trials on SLE using new biologicals, based on newly understood SLE mechanisms, have shown promising results. Based on what we currently know about its pathogenesis, it is tempting to speculate how these new therapies may affect the management of NPSLE patients. This article provides a comprehensive and critical review of the literature on the epidemiology, pathophysiology, diagnosis, and management of NPSLE. We describe the most common pharmacological treatments used in NPSLE, based on both a literature search and our expert opinion. The extent to which new drugs in the advanced development of SLE, or the blockade of new targets, may impact future treatment of NPSLE will also be discussed.
引自：César Magro-Checa, Elisabeth J. Zirkzee, Tom W. Huizinga,et al.Management of Neuropsychiatric Systemic Lupus Erythematosus: Current Approaches and Future Perspectives.Drugs. 2016; 76: 459–483.Published online 2016 Jan 26. doi: 10.1007/s40265-015-0534-3
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