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Abstract：
Neuropsychiatric systemic lupus erythematosus (NPSLE) refers to the neurological and psychiatric manifestations of systemic lupus erythematosus (SLE), which remain poorly understood yet often have a profound effect on the lives of afflicted patients. The aim of this study is to synthesize the available information on the pathogenesis, diagnostics, management, and prognosis of this disease. Our hope is to increase awareness and call for further investigations that may optimize NPSLE patient outcomes and quality of life. We performed a literature review following the Preferred Reporting Items for Systematic reviews and Meta-Analyses guidelines, resulting in 11 studies of inclusion. Within each study, we extracted data on epidemiologic factors, diagnostics, therapeutic modalities, and prognosis for each neuropsychiatric condition. The most widely discussed neuropsychiatric manifestations of SLE based on the American College of Rheumatology (ACR) classifications included status epilepticus (SE) and seizures, transverse myelitis (TM), and cognitive dysfunction. SE and TM had a prevalence of 1-2%, while cognitive dysfunction was nearly 38%. Diagnostics varied depending on symptom presentation but often included brain magnetic resonance imaging (MRI) and antibody testing. Treatment for NPSLE is still widely understudied, but concurrent treatment with immunosuppressants and anti-inflammatories for symptom control and more targeted immunotherapies based on the specific condition is often effective. Prognosis is highly symptom dependent, ranging from a 12.5% one-year mortality in SE and seizure patients to near resolution of symptoms in certain presentations including idiopathic intracranial hypertension and cerebellar ataxia. Further studies are needed to better understand the pathophysiology, diagnostics, and effective therapeutic measures for NPSLE. The severity of these manifestations and generally poor prognosis highlight the need for more research to accurately diagnose and treat this disease. While there is still little data available, this literature review serves to provide updated context on this condition.
摘要：
神经精神系统性红斑狼疮（NPSLE）是指系统性红斑狼疮（SLE）的神经和精神表现，人们仍然知之甚少，但往往对患病患者的生活产生深远的影响。这项研究的目的是综合有关发病机理的现有信息，诊断,管理,和这种疾病的预后。我们的希望是提高认识，并呼吁进一步的调查，可以优化NPSLE患者的结果和生活质量。我们根据系统评价和荟萃分析指南的首选报告项目进行了文献综述，导致11项纳入研究。在每项研究中，我们提取了流行病学因素的数据，诊断,治疗方式，以及每种神经精神疾病的预后。根据美国风湿病学会（ACR）分类，讨论最广泛的SLE神经精神表现包括癫痫持续状态（SE）和癫痫发作。横贯性脊髓炎(TM),和认知功能障碍。SE和TM的患病率为1-2%，而认知功能障碍接近38%。诊断根据症状表现而有所不同，但通常包括脑磁共振成像（MRI）和抗体测试。NPSLE的治疗仍被广泛研究，但同时使用免疫抑制剂和抗炎药进行症状控制和根据具体情况进行更有针对性的免疫疗法通常是有效的.预后高度依赖于症状，从SE和癫痫患者的一年死亡率为12.5%，到某些表现包括特发性颅内高压和小脑共济失调的症状几乎消失。需要进一步的研究来更好地了解病理生理学，诊断,和NPSLE的有效治疗措施。这些表现的严重程度和通常不良的预后凸显了需要更多的研究来准确诊断和治疗这种疾病。虽然可用的数据仍然很少，这篇文献综述有助于提供这种情况的最新背景。
