Management of Neuropsychiatric Systemic Lupus Erythematosus: Current Approaches and Future Perspectives.
Management of Neuropsychiatric Systemic Lupus Erythematosus: Current Approaches and Future Perspectives.
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DOI：
10.1007/s40265-015-0534-3
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发表时间：
2016-03
期刊：
影响因子：
11.5
通讯作者：
Steup-Beekman GM
中科院分区：
文献类型：
--
作者：
Magro-Checa C;Zirkzee EJ;Huizinga TW;Steup-Beekman GM
神经精神系统性红斑狼疮（NPSLE）是一个通用的定义，是指一系列的神经和精神症状直接相关的系统性红斑狼疮（SLE）。NPSLE包括涉及中枢和外周神经系统的异质性和罕见的神经精神（NP）表现。由于缺乏金标准，将NP症状归因于SLE是一项临床挑战，必须严格排除任何其他潜在原因。在急性环境中，这些患者的管理与其他表现出相同NP表现的非SLE受试者没有区别。之后，必须根据症状的表现和严重程度开始个性化的治疗策略。NPSLE的临床试验很少，大部分数据来自病例系列和病例报告。高剂量糖皮质激素和静脉注射环磷酰胺仍然是出现严重症状（被认为反映了炎症或潜在的自身免疫过程）的患者的基石。如果未达到应答，可使用利妥昔单抗、静脉注射免疫球蛋白或血浆置换。当患者出现轻度至中度NP表现，或需要维持治疗时，可考虑硫唑嘌呤和霉酚酸酯。当症状被认为反映了血栓形成的潜在过程时，抗凝和抗血小板药物是治疗的主要手段，特别是当存在抗磷脂抗体或抗磷脂综合征时。最近的试验SLE使用新的生物制剂，根据新了解的SLE机制，已显示出可喜的成果。根据我们目前对其发病机制的了解，推测这些新疗法可能如何影响NPSLE患者的治疗是很诱人的。本文对NPSLE的流行病学、病理生理学、诊断和治疗方面的文献进行了全面和批判性的综述。基于文献检索和我们的专家意见，我们描述了NPSLE中最常见的药物治疗。在何种程度上的新药在先进的发展系统性红斑狼疮，或封锁新的目标，可能会影响未来的治疗NPSLE也将进行讨论。
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 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.