Department of Immunology, University Hospital of Zurich, Zurich, Switzerland.
Medignition Healthcare Innovations, Zurich, Switzerland.
Lupus. 2021 Sep;30(10):1565-1576. doi: 10.1177/09612033211025636. Epub 2021 Jun 21.
Systemic lupus erythematosus (SLE) is a systemic autoimmune disease associated with neuro-psychiatric (NP) manifestations. Frequency and patterns of neuro-psychiatric systemic lupus erythematosus (NPSLE) vary substantially between patients. We conducted a systematic review (SR) of the literature and examined prevalence and characteristics of NPSLE in the Swiss SLE cohort study (SSCS).
The SR search was performed between January 1999 and January 2020. We included prospective/cross-sectional studies focusing on NPSLE. We secured study characteristics, cohort compositions and frequencies of NP manifestations, assessed heterogeneity across reports and investigated sources of variation using meta-regression models. Regarding the SSCS, we reviewed all patients included and classified NP manifestations.
The SR searches identified 530 studies. We included 22 studies in our meta-analysis, the mean frequency of NPSLE ranged from 10.6% to 96.4%. The frequency of NPSLE in the SSCS was 28.1%. Severe events including cerebrovascular insults, seizures and psychosis appeared in 7.1%, 5.3% and 6.5% respectively. There was a linear relationship between duration of SLE and cumulative incidence of NPSLE.
The spectrum of NPSLE is very broad. The diagnostic work-up and rates of reported manifestations varied substantially across studies. We call for concerted efforts and consensus regarding definitions of NPSLE that will facilitate accurate diagnosis and attribution to SLE, particularly with a view to timely intervention and patient outcomes.
系统性红斑狼疮（SLE）是一种与神经精神（NP）表现相关的系统性自身免疫性疾病。NP 系统性红斑狼疮（NPSLE）在患者之间的频率和模式有很大差异。我们对文献进行了系统评价（SR），并检查了瑞士 SLE 队列研究（SSCS）中 NPSLE 的患病率和特征。
SR 搜索于 1999 年 1 月至 2020 年 1 月进行。我们纳入了专注于 NPSLE 的前瞻性/横断面研究。我们获取了研究特征、队列组成和 NP 表现的频率，评估了报告之间的异质性，并使用荟萃回归模型调查了变异的来源。关于 SSCS，我们回顾了所有纳入的患者并对 NP 表现进行了分类。
SR 搜索确定了 530 项研究。我们纳入了 22 项荟萃分析研究，NPSLE 的平均频率范围为 10.6%至 96.4%。SSCS 中 NPSLE 的频率为 28.1%。严重事件包括脑血管损伤、癫痫发作和精神病，发生率分别为 7.1%、5.3%和 6.5%。SLE 持续时间与 NPSLE 的累积发生率之间存在线性关系。
NPSLE 的范围非常广泛。诊断工作和报告的表现率在研究之间有很大差异。我们呼吁就 NPSLE 的定义达成一致努力和共识，这将有助于准确诊断和归因于 SLE，特别是考虑到及时干预和患者结局。
