Neuropsychiatric Lupus Erythematosus: Future Directions and Challenges; a Systematic Review and Survey.

神经精神性红斑狼疮: 未来的方向和挑战; 系统回顾和调查。

  • 影响因子:1.35
  • DOI:10.6061/clinics/2020/e1515
  • 作者列表:"Zhang Y","Han H","Chu L
  • 发表时间:2020-04-17

:This study aimed to systematically review neuropsychiatric lupus erythematosus (NPSLE) and establish a simplified diagnostic criterion for NPSLE. Publications from 1994 to 2018 in the database (Wanfang data (http://www.wanfangdata.com.cn/index.html) and China National Knowledge Internet (http://www.cnki.net)) were included. In total, 284 original case reports and 24 unpublished cases were collected, and clinical parameters were analyzed. An attempt was made to develop a set of simplified diagnostic criteria for NPSLE based on cases described in the survey and literature; moreover, and pathophysiology and management guidelines were studied. The incidence rate of NPSLE was estimated to be 12.4% of SLE patients in China. A total of 408 NPSLE patients had 652 NP events, of which 91.2% affected the central nervous system and 8.8% affected the peripheral nervous system. Five signs (manifestations, disease activity, antibodies, thrombosis, and skin lesions) showed that negative and positive predictive values were more than 70%, included in the diagnostic criteria. The specificity, accuracy, and positive predictive value (PPV) of the revised diagnostic criteria were significantly higher than those of the American College of Rheumatology (ACR) criteria (χ2=13.642, 15.591, 65.010, p<0.001). The area under the curve (AUC) for revised diagnostic criteria was 0.962 (standard error=0.015, 95% confidence intervals [CI] =0.933-0.990), while the AUC for the ACR criteria was 0.900 (standard error=0.024, 95% CI=0.853-0.946). The AUC for the revised diagnostic criteria was different from that for the ACR criteria (Z=2.19, p<0.05). Understanding the pathophysiologic mechanisms leading to NPSLE is essential for the evaluation and design of effective interventions. The set of diagnostic criteria proposed here represents a simplified, reliable, and cost-effective approach used to diagnose NPSLE. The revised diagnostic criteria may improve the accuracy rate for diagnosing NPSLE compared to the ACR criteria.


: 本研究旨在系统回顾神经精神性红斑狼疮 (NPSLE) 并建立简化的NPSLE诊断标准。数据库中1994年至2018年的出版物 (万方数据 ( http://www.wanfangdata.com.cn/index.html ) 和中国知识互联网 ( http://www.cnki.net )) 包括在内。共收集284例原始病例报告和24例未发表病例,并对临床参数进行分析。根据调查和文献中描述的病例,尝试开发一套简化的NPSLE诊断标准; 此外,还研究了病理生理学和管理指南。NPSLE在中国SLE患者中的发病率估计为12.4%。共有408例NPSLE患者发生652例NP事件,其中91.2% 例影响中枢神经系统,8.8% 例影响外周神经系统。5个体征 (表现、疾病活动、抗体、血栓形成、皮损) 显示阴性和阳性预测值均超过70%,纳入诊断标准。修订后的诊断标准的特异性、准确性、阳性预测值 (PPV) 均明显高于美国风湿病学会 (ACR) 标准 (χ2 2 = 13.642、15.591、65.010,p<0.001)。修订后的诊断标准的曲线下面积 (AUC) 为0.962 (标准误差 = 0.015,95% 置信区间 [CI] = 0.933-0.990),而ACR标准的AUC为0.900 (标准误差 = 0.024,95% CI = 0.853-0.946)。修订后的诊断标准的AUC与ACR标准的AUC不同 (Z = 2.19,p<0.05)。了解导致NPSLE的病理生理机制对于评估和设计有效的干预措施至关重要。这里提出的一组诊断标准代表了一种用于诊断NPSLE的简化、可靠和具有成本效益的方法。与ACR标准相比,修订的诊断标准可以提高诊断NPSLE的准确率。



作者列表:["Apivatthakakul A","Kunavisarut P","Rothova A","Pathanapitoon K"]

METHODS::Purpose: To report on ocular Vogt-Koyanagi-Harada (VKH)-like syndrome under vemurafenib treatment for metastatic melanoma.Design: A case report.Method: Description of clinical and imaging manifestations including fundus photography, fluorescein, and indocyanine green angiography.Results: A 46-year-old Thai female was diagnosed with metastatic melanoma of the skin and had been treated with multiple surgical excisions, radiotherapy, and vemurafenib (initial dose 480 mg orally twice daily, subsequently increased to maximum dose of 960 mg twice daily). After 6 months of vemurafenib use, she complained of bilateral redness and photophobia and was diagnosed with bilateral anterior uveitis, which was topically treated. Two weeks later, her visual acuity (VA) sharply deteriorated to 20/80 and counting fingers. Ocular examination at that stage stronly resembled acute VKH disease. She exhibited intraocular inflammation, and her fundus examination revealed bilateral optic disc swelling and serous retinal detachment. Fluorescein angiogram showed disc leakage and multiple pinpoint hyperfluorescence leakage spots and indocyanine green demonstrated multiple hypofluorescent spots. Oral prednisolone 30 mg/day was commenced while vemurafenib medication was ceased. Three weeks later, her vision improved, and serous retinal detachment subsided. However, her cutaneous melanoma recurred.Conclusions: Vemurafenib, a potential adjunct treatment for metastatic melanoma, was complicated by the development of panuveitis, papillitis, and multiple serous detachments. These ocular symptoms were similar to the presentation of acute VKH syndrome.

翻译标题与摘要 下载文献
作者列表:["Crow YJ","Shetty J","Livingston JH"]

METHODS::Comprehensive reviews of the clinical characteristics and pathogenesis of Aicardi-Goutières syndrome (AGS), particularly its contextualization within a putative type I interferonopathy framework, already exist. However, recent reports of attempts at treatment suggest that an assessment of the field from a therapeutic perspective is warranted at this time. Here, we briefly summarize the neurological phenotypes associated with mutations in the seven genes so far associated with AGS, rehearse current knowledge of the pathology as it relates to possible treatment approaches, critically appraise the potential utility of therapies, and discuss the challenges in assessing clinical efficacy. WHAT THIS PAPER ADDS: Progress in understanding AGS disease pathogenesis has led to the first attempts at targeted treatment. Further rational therapies are expected to become available in the short- to medium-term.

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翻译标题与摘要 下载文献
作者列表:["Takayama K","Obata H","Takeuchi M"]

METHODS::Purpose: To report the efficacy of adalimumab in a case of chronic Vogt-Koyanagi-Harada (VKH) disease refractory to conventional corticosteroids and immunosuppressive therapy and complicated by central serous chorioretinopathy (CSC).Case report: A 66-year-old woman diagnosed with VKH was treated with intravenous corticosteroids followed by oral corticosteroids and cyclosporine. However, systemic corticosteroids could not be tapered because of recurrent ocular inflammation and systemic complications (diabetes mellitus, moon face, bone weakness), while CSC appeared in both eyes. A diagnosis of chronic VKH resistant to medications complicated by corticosteroid-induced CSC was made. Systemic corticosteroids and cyclosporine were tapered and adalimumab initiated. Bilateral ocular inflammation and CSC were gradually reduced and visual acuity improved without any adverse effect. Twelve months after starting adalimumab monotherapy, no signs of active VKH and CSC were present.Conclusions: Adalimumab is one of the effective therapeutic options for refractory VKH disease complicated with corticosteroid-induced adverse effects.