Neuropsychiatric manifestations of systemic lupus erythematosus (SLE) are common and frequently associated with a substantial negative impact on health outcomes. The pathogenesis of neuropsychiatric SLE (NPSLE) remains largely unknown, but a single pathogenic mechanism is unlikely to be responsible for the heterogeneous array of clinical manifestations, and a combination of inflammatory and ischaemic mechanistic pathways have been implicated. Currently, valid and reliable biomarkers for the diagnosis of NPSLE are lacking, and differentiating NPSLE from nervous system dysfunction not caused by SLE remains a major challenge for clinicians. However, correct attribution is essential to ensure timely institution of appropriate treatment. In the absence of randomized clinical trials on NPSLE, current treatment strategies are derived from clinical experience with different therapeutic modalities and their efficacy in the management of other manifestations of SLE or of neuropsychiatric disease in non-SLE populations. This Review describes recent advances in the understanding of NPSLE that can inform diagnosis and management, as well as unanswered questions that necessitate further research.

系统性红斑狼疮 （SLE） 的神经精神表现很常见，并且经常与对健康结果的重大负面影响有关。神经精神 SLE （NPSLE） 的发病机制在很大程度上仍然未知，但单一的致病机制不太可能导致临床表现的异质性，并且涉及炎症和缺血机制途径的结合。目前，缺乏用于诊断 NPSLE 的有效且可靠的生物标志物，区分 NPSLE 与非 SLE 引起的神经系统功能障碍仍然是临床医生面临的主要挑战。然而，正确的归因对于确保及时进行适当的治疗至关重要。在缺乏关于 NPSLE 的随机临床试验的情况下，目前的治疗策略来自不同治疗方式的临床经验及其在治疗非 SLE 人群中 SLE 或神经精神疾病的其他表现的疗效。本综述描述了对 NPSLE 的理解的最新进展，这些进展可以为诊断和管理提供信息，以及需要进一步研究的未回答的问题。