Tay Sen Hee, Mak Anselm
Department of Medicine, Yong Loo Lin School of Medicine, National University of Singapore.
Division of Rheumatology, Department of Medicine, National University Hospital, National University Health System, Singapore.
Rheumatology (Oxford). 2017 Apr 1;56(suppl_1):i14-i23. doi: 10.1093/rheumatology/kew338.
Neurological and psychiatric syndromes, collectively referred to as NPSLE, occur frequently in SLE. The frequency of NPSLE varies from 21 to 95%; however, only 13-38% of neuropsychiatric (NP) events could be attributable to SLE in the NPSLE SLICC inception cohort. This variability in the frequency of NPSLE is attributable to the low specificity of the ACR case definitions for SLE-attributed NP syndromes, inclusion of minor NP events in the ACR nomenclature, difficulty in ascertainment of NP events and diverse experience of rheumatologists in the clinical assessment of NP events. Making the correct and early attribution of NP events to SLE is important to institute appropriate immunosuppressive treatment for favourable outcomes. Various attribution models using composite decision rules have been developed and used to ascribe NP events to SLE. This review will focus on the various clinical presentations, diagnostic work-up and attributions of the common NPSLE syndromes, including other NP events not included in the ACR nomenclature but which have come to attention in recent years.
神经精神综合征统称为神经精神性狼疮（NPSLE），在系统性红斑狼疮（SLE）中经常出现。NPSLE的发生率在21%至95%之间；然而，在NPSLE的SLICC初始队列中，只有13%至38%的神经精神（NP）事件可归因于SLE。NPSLE发生率的这种变异性归因于美国风湿病学会（ACR）关于SLE相关NP综合征病例定义的低特异性、ACR命名法中纳入了轻微NP事件、NP事件难以确定以及风湿病学家在NP事件临床评估中的不同经验。对NP事件正确且早期归因于SLE对于采取适当的免疫抑制治疗以获得良好结局很重要。已经开发了各种使用综合决策规则的归因模型并用于将NP事件归因于SLE。本综述将重点关注常见NPSLE综合征的各种临床表现、诊断检查和归因，包括ACR命名法中未包括但近年来受到关注的其他NP事件。
