Department of Rheumatology, St. Vincent's Hospital, Melbourne, Australia.
Department of Rheumatology, St. Vincent's Hospital, Melbourne, Australia; Department of Medicine, The University of Melbourne, Australia; Department of Rheumatology, The Royal Melbourne Hospital, Australia.
Semin Arthritis Rheum. 2021 Feb;51(1):49-71. doi: 10.1016/j.semarthrit.2020.12.004. Epub 2020 Dec 17.
The neurological and psychiatric manifestations of systemic lupus erythematosus (NPSLE) are a heterogeneous group of conditions with variable clinical presentation and significant morbidity and mortality.
Our aim was to comprehensively assess and present the evidence for treatments used in the management of inflammatory NPSLE.
Medline, Embase, CINHAL and Cochrane CENTRAL were searched from 1990 to end of March 2019 using key words that related to NPSLE and treatment. Included studies comprised clinical trials, observational studies or case series with ≥5 patients and sufficient data related to treatment and outcome in NPSLE patients.
There were 7222 studies identified in the search, of which 90 were included in the review. There was a notable paucity of clinical trials, with only two randomised controlled trials and one pilot study. Treatment categories included corticosteroids (14 studies), cyclophosphamide (18 studies), synthetic DMARDs (7 studies), biologic therapies (14 studies), therapeutic plasma exchange (6 studies), intravenous immunoglobulin (2 studies), autologous stem cell transplant (3 studies), other therapies (8 studies), combination therapies (6 studies), studies with grouped outcome data (5 studies) and observational studies with therapy-specific associations (7 studies). Corticosteroids are accepted as first line treatment in NPSLE and there is low-moderate evidence supporting their benefit. Moderate evidence, based on consistent data in numerous studies and some trial data, supports the use of cyclophosphamide in the treatment of NPSLE. Limited data support some synthetic DMARDs such as mycophenolate, azathioprine and intrathecal methotrexate. In refractory disease, low-moderate evidence supports rituximab therapy and limited evidence supports benefit following autologous stem cell transplant. Regarding adjuvant treatments, limited evidence favours addition of plasma exchange, intravenous immunoglobulin and hydroxychloroquine. There exists very limited data for other therapies.
There are multiple therapeutic options for the management of inflammatory NPSLE including systemic, biologic and interventional therapies; however, currently there is a paucity of high-quality trial data to guide firm recommendations. In order to better understand the optimal treatment of NPSLE and its different subtypes, further well-designed clinical trials are needed.
系统性红斑狼疮（SLE）的神经和精神表现（NPSLE）是一组异质性疾病，临床表现多样，发病率和死亡率高。
我们旨在全面评估和介绍治疗炎症性 NPSLE 的证据。
使用与 NPSLE 和治疗相关的关键词，从 1990 年到 2019 年 3 月底，在 Medline、Embase、CINHAL 和 Cochrane CENTRAL 中进行了搜索。纳入的研究包括临床试验、观察性研究或有≥5 例患者的病例系列研究，且有足够的数据与 NPSLE 患者的治疗和结局相关。
在搜索中发现了 7222 项研究，其中 90 项被纳入综述。仅有两项随机对照试验和一项试点研究，临床试验明显不足。治疗类别包括皮质类固醇（14 项研究）、环磷酰胺（18 项研究）、合成 DMARDs（7 项研究）、生物制剂（14 项研究）、治疗性血浆置换（6 项研究）、静脉注射免疫球蛋白（2 项研究）、自体干细胞移植（3 项研究）、其他治疗方法（8 项研究）、联合治疗（6 项研究）、汇总结局数据的研究（5 项研究）和特定治疗关联的观察性研究（7 项研究）。皮质类固醇被认为是 NPSLE 的一线治疗方法，有低到中度证据支持其疗效。基于大量研究中一致的数据和一些试验数据，中等证据支持环磷酰胺治疗 NPSLE。有限的数据支持一些合成 DMARDs，如霉酚酸、硫唑嘌呤和鞘内甲氨蝶呤。在难治性疾病中，低到中度证据支持利妥昔单抗治疗，有限证据支持自体干细胞移植后的获益。关于辅助治疗，有限的证据支持添加血浆置换、静脉注射免疫球蛋白和羟氯喹。其他治疗方法的数据非常有限。
对于炎症性 NPSLE 的治疗有多种治疗选择，包括全身治疗、生物制剂和介入治疗；然而，目前缺乏高质量的临床试验数据来指导确切的推荐。为了更好地了解 NPSLE 及其不同亚型的最佳治疗方法，需要进一步进行精心设计的临床试验。
