小剂量利妥昔单克隆抗体治疗重症狼疮的疗效和安全性分析

正系统性红斑狼疮(systemic lupus erythematosus,SLE)是一类机体免疫系统功能异常,多种自身抗体产生,常导致多器官、多系统受累的自身免疫性疾病。神经精神狼疮(neuropsychiatric systemic lupus erythematosus,NPSLE)、重度狼疮性肾炎、重度血小板减少和重度肺动脉高压(pulmonary arterial     

视神经脊髓炎合并系统性红斑狼疮及干燥综合征1例报告

<正>神经精神性狼疮(neuropsychiatric systemic lupus erythematosus,NPSLE)是系统性红斑狼疮(systemic lupus erythematosus,SLE)常见的并发症之一,有报道称其发生率高达14%~81%[1],并可作为SLE的首发症状。典型的SLE中枢神经系统受累包括癫痫、抑郁、多发性单神经炎,但也可出现横贯性脊髓炎、脱髓鞘或颅神经受累等少见临床表现。以中枢神经系统损害为首发症状的SLE容易被误诊、延误治     

静息态和任务态fMRI在神经精神性狼疮中的研究进展

系统性红斑狼疮(systemic lupus erythematosus,SLE)累及神经系统时称为神经精神性狼疮(neuropsychiatric systemic lupus erythematosus,NPSLE),是患者发病和死亡的重要原因,且严重影响患者的生活质量,早期诊断、早期临床干预是提高患者预后的关键。功能性磁共振成像(functional magnetic resonance imaging,fMRI)技术不仅能早期发现SLE患者脑功能异常,且在揭示其发病机制方面具有一定优势。笔者就静息态和任务态fMRI在NPSLE中的应用研究及进展予以综述。     

误诊为短暂性脑缺血发作的狼疮脑病1例报道

<正>系统性红斑狼疮(systemic lupus erythematosus,SLE)是一种复杂的自身免疫性结缔组织病,常好发于青年女性,累及如心肺、神经、肾脏、消化等多系统。狼疮脑病也称为神经精神狼疮(neuropsythiatric systemic lupus erythematosus,NPSLE),是该病的严重并发症,有研究表明神经系统症状的介入成为住院患者的独立危险因素及警示标志,可能提示更     

神经精神狼疮治疗进展

神经精神狼疮(neuropsychiatric systemic lupus erythematosus,NPSLE)是系统性红斑狼疮(systemic lupus erythematosus,SLE)引起的中枢、周围和自主神经系统和精神系统的一系列症状,可作为首发症状在发病5年内出现,也可在SLE复发及激素治疗减量过程中发生,发病率高,在中国SLE人群中发病率约19%,而国外报告此比例可高达 46%[1].可有头痛、性格改变、记忆力减退、脑血管意外、昏迷、癫痫持续状态等多种表现,正确的治疗利于改善预后,但国内相关治疗方法的报道较少,现就近年国外治疗神经精神狼疮的进展做一综述.     

系统性红斑狼疮患者血清IgG水平正常及升高与相关免疫学实验室指标的相关分析

目的 探讨系统性红斑狼疮患者(systemic lupus erythematosus,SLE)中免疫球蛋白G(immunoglobulin G,IgG)正常与IgG升高的患者,其抗核抗体(antinuclear antibody,ANA)滴度、抗双链DNA(double-strand DNA,ds-DNA)抗体及抗可...     

贝利尤单抗治疗重症神经精神性狼疮患者一例并文献复习

系 统 性 红 斑 狼 疮(systemic lupus erythematosus,SLE)是一种多系统累及的自身免疫性疾病,神经精神性狼疮(neuropsychiatric SLE, NPSLE)是其较为严重的并发症,可出现在SLE病程中的任何阶段,大部分发生在SLE初发阶段,是SLE预后不良的主要因素之一.NPS...     

神经精神狼疮患者的护理现状

神经精神狼疮(neuropsychitric disorders in systemic lupus erythematosus NPSLE)俗称狼疮脑病,是一种累及多系统、多器官并有多种自身抗体出现的自身免疫性疾病.我国发病率约为75/10万.SLE累及神经系统,尤以累及脑多见,称为神经精神狼疮(NPSLE).NPSLE的发病率各家报道不同,从19.4%～91%,可能与各个研究选取的诊断标准及研究对象的不同有关.其病情复杂,临床表现变化多样而迅速,对护理工作带来一定难度.本文针对这一现象参考近期国内外护理杂志就其常见症状及护理特点做一综述.     

抗β2-GPI抗体与狼疮性肾炎的相关性研究

系统性红斑狼疮(systemic lupus erythematosus,SLE)是继发性抗磷脂综合征(antiphospholipid syndrome,APS)的典型代表,其抗β2-GPI抗体阳性率高达65％。狼疮性肾炎(lupus nephritis,LN)是SLE累及肾脏而引起的肾脏炎症,SLE患者罹患者约2／3。本文探讨了血清抗β2-GPI抗体水平与LN的相关性,报道如下。     

护理程序在神经精神狼疮病人护理中的应用

系统性红斑狼疮（systemic lupus erythematosus,SLE）是一种原因不明的慢性复发一缓解性的自身免疫性疾病,以多种自身抗体导致不同靶器官的损害为特点。以神经精神症状为表现,即神经精神狼疮（neuropsychiatric,NPSLE）,其发病率高达14％-75％。由于种族、年龄、性别,尤其是诊断标准使用的差异,NPSLE占SLE的比例大致在40％～50％,并具有极高的病死率和致残率。国内外风湿病学界却对NPSLE的治疗缺乏系统性研究,多以经验治疗为主。     

Echolalia as a novel manifestation of neuropsychiatric systemic lupus erythematosus

"That tongue of yours, by which I have been tricked, shall have its power curtailed and enjoy the briefest use of speech." With these words, Hera, of Greek mythology, deprived the nymph Echo of spontaneous speech, constraining her instead to merely repeating the words of others. Echolalia, which derives from the word "echo," is disordered speech in which an individual persistently repeats what is heard. Echolalia has been described in patients with a number of neuropsychiatric illnesses including autism and Tourette's syndrome. Neuropsychiatric systemic lupus erythematosus (NPSLE) is a heterogeneous disease with protean manifestations that may occur in approximately 25% to 50% of patients with systemic lupus erythematosus (SLE). Although the most common manifestations include cognitive dysfunction (50%) and seizures (20%), NPSLE may also present as peripheral neuropathy (15%), psychosis (10%), or other central nervous system abnormalities. We report the case of a 57-year-old woman with SLE and echolalia.     

Anti-histones antibodies in systemic lupus erythematosus: prevalence and frequency in neuropsychiatric lupus

To investigate the specificity, sensitivity, and concomitant presence of antibodies against histones (H), histone H1 (H1), and histone H3 (H3) in patients with systemic lupus erythematosus (SLE) and analyze their association with SLE. Serum IgG anti-histones antibodies were detected by enzyme-linked immunosorbent assay in 144 SLE patients consisting of 24 neuropsychiatric lupus (NPSLE), 65 lupus nephritis (LN), and 55 SLE, 100 other rheumatic diseases patients, as well as 40 healthy controls. Clinical and biological parameters of the patients were also evaluated. Anti-H, anti-H1, and anti-H3 antibodies yielded a sensitivity of approximately 33% and a specificity of more than 93% for SLE, which was comparable to that found for anti-double-stranded DNA (anti-dsDNa) antibodies. More significantly, anti-histone antibody is found in approximately 50% of patients with NPSLE compared with LN. Moreover, the titers of anti-histones antibodies of NPSLE patients were significantly higher than that of patients with SLE and LN. The sequential analysis revealed a close correlation of anti-H and anti-H1 antibodies with SLE disease activity. There was an approximate 30% positive rate of anti-histones antibodies in 144 SLE patients lacking anti-nucleosome, anti-mDNA, anti-Sm, and anti-dsDNA antibodies. Antibodies to histones H1 and H3 are markers with high specificity of 93.6-96.4% for SLE. The anti-histone antibody markers are prevalent in approximately 50% of NPSLE. Furthermore, there was a strong correlation with SLE disease activity index and levels of antibodies to histones and H1.     

并发神经精神狼疮的儿童狼疮肾炎34例临床分析

目的:分析并发神经精神性狼疮(NPSLE)的儿童狼疮性肾炎(LN)患者的临床特点。方法:对122例首次诊断为NPSLE合并LN的住院患者进行回顾性研究,收集其临床资料,比较儿童起病组(n=34)与成年起病组(n=88)的临床特点,包括:性别、系统性红斑狼疮(SLE)的病程、临床表现、实验室检查等。结果:儿童起病组SLE病程较成年起病组短(P0.05);儿童起病组NPSLE以痫性发作为主(70.6%),而成年起病组以精神症状为主(52.3%),两组间存在显著性差异。成年起病组发生白细胞减少的比例明显高于儿童起病组(P0.05)。结论:儿童起病的NPSLE合并LN患者的SLE病程、NPSLE临床类型、发生白细胞减少的比例与成年起病者不同,对不同年龄起病的NPSLE合并LN患者应给予不同的治疗策略。     

The therapeutic potential of targeting anti-Ribosomal-P antibody in treating SLE patients with depression

Neuropsychiatric systemic lupus erythematosus (NPSLE) involves the central and peripheral nervous system. The pathogenesis of NPSLE is poorly understood. The condition is recognised as one of the major causes of mortality in SLE patients. The main autoantibody population associated with NPSLE is directed to ribosomal-phosphoproteins (anti-Ribos.P), which can bind the limbic area of mouse brain and penetrate into neuronal cells in vitro. Intracerebroventricular injection of these antibodies, induce experimental depression-like models in naive mice. Based on these observations, targeting anti-Ribos.P antibodies may be a new therapeutic approach to treat NPSLE patients with depression.     

The therapeutic potential of targeting anti-Ribosomal-P antibody in treating SLE patients with depression

Neuropsychiatric systemic lupus erythematosus (NPSLE) involves the central and peripheral nervous system. The pathogenesis of NPSLE is poorly understood. The condition is recognised as one of the major causes of mortality in SLE patients. The main autoantibody population associated with NPSLE is directed to ribosomal-phosphoproteins (anti-Ribos.P), which can bind the limbic area of mouse brain and penetrate into neuronal cells in vitro. Intracerebroventricular injection of these antibodies, induce experimental depression-like models in naive mice. Based on these observations, targeting anti-Ribos.P antibodies may be a new therapeutic approach to treat NPSLE patients with depression.     

抗核小体抗体对系统性红斑狼疮的诊断价值

目的 研究抗核小体抗体(AnuA)对系统性红斑狼疮(SLE)诊断的敏感性和特异性.探讨AnuA在SLE诊治中的作用和意义.方法 采用免疫印迹法检测90例系统性红斑狼疮患者血清,40例其他风湿性疾病患者血清和35例健康对照血清中的AnuA及其他自身抗体指标,并将AnuA与疾病活动度、抗双链DNA抗体(抗-dsDNA)、抗-Sm补体等指标进行比较.结果 AnuA在SLE及其他风湿病对照组的阳性率分别为AnuA 72%和10%;抗-ds DNA 65.3%和1%,抗-Sm 22.5%和0.对SLE诊断的敏感性和特异性分别为69.2%和96%.AnuA与疾病活动度,SLE DAI评分,抗-ds DNA,补体C3水平和血沉有相关性.结论 AnuA在SLE中敏感性较高,是诊断SLE和了解狼疮活动度的良好指标.     

神经精神性狼疮认知功能改变机制及结构和功能MRI研究进展

25%~75%的系统性红斑狼疮患者中存在中枢神经系统受累,尤其是脑,可产生各种神经精神症状,而且有证据表明嗅觉和认知功能改变可能早于神经精神症状的发生,因此有助于对狼疮脑病的早期诊断。近年来,随着各种功能MRI技术的发展,对系统性红斑狼疮的诊断、评估以及疾病的发生发展有重要价值。作者就系统性红斑狼疮患者在嗅觉和认知方面的改变,以及结构和功能MRI在其中的应用展开综述。     

基于肽抗原的间接酶联免疫吸附法检测抗水通道蛋白4抗体在神经精神狼疮中的临床意义

目的：建立抗水通道蛋白4（AQ-4）抗体的检测方法,评估抗AQ-4抗体在神经精神狼疮（NPSLE）中的临床意义。方法：用3段AQ-4细胞外肽段包板,建立基于肽抗原的间接酶联免疫吸附（ELISA）检测方法,检测49例健康人、54例其他风湿病患者（32例多肌炎/皮肌炎,10例干燥综合征和12例类风湿关节炎患者）、105例系统性红斑狼疮（SLE）患者和103例NPSLE患者的血清中的抗AQ-4抗体;检测22例非风湿病患者、31例SLE患者、96例NPSLE、27例合并结核感染的SLE患者和8例隐球菌脑膜炎患者的脑脊液标本中抗AQ-4抗体。结果：以健康人（对照组）的平均光密度（OD）＋3标准差（SD）为临界值,血清标本阳性率在其他风湿病组为1.8%（1/54）;SLE组为14.3%（15/105）;NPSLE组为22.3%（23/103）。脑脊液标本阳性率在对照组为0%;SLE组为12.9%（4/31）;NPSLE组为29.2%（28/96）;SLE合并结核性脑膜炎者为37%（10/27）;SLE合并隐球菌脑膜炎者为0%（0/8）。SLE组合并NPSLE组患者的血清和脑脊液中的抗AQ-4抗体显著增加（P〈0.01）,SLE组血清和脑脊液中的抗AQ-4抗体水平相似（P〉0.05）。NPSLE组脑脊液中的抗体检出率（29.2%）较血清中抗体检出率（22.3%）增高（P〈0.01）。在SLE合并TB组的脑脊液中,抗AQ-4抗体的检出显著增高（P〈0.01）。结论：抗AQ-4抗体在SLE和NPSLE患者的血清和脑脊液中显著升高,尤其是脑脊液中。SLE合并结核性脑膜炎患者的抗AQ-4抗体显著升高,提示抗AQ-4抗体是狼疮合并结核性脑病的易患因素。     

狼疮带试验对诊断系统性红斑狼疮的意义

目的　探讨狼疮带试验 (LBT)在系统性红斑狼疮 (SLE)诊断中的意义。方法 :对 15 0例SLE患者和 5 0例非SLE患者分别检测血清ANA、ds-DNA、补体、免疫球蛋白、尿素氮、肌酐、尿蛋白定性及定量、肾脏组织病理、LBT免疫成分。结果　LBT在SLE诊断中的敏感性为 71%、特异性为 84 %、阳性预测值为 93%、阴性预测值为 4 8% ,LBT阳性与肾损害及狼疮活动关系密切。结论　LBT是SLE的诊断的一项重要辅助指标     

抗核抗体、免疫球蛋白及补体联合检测对狼疮性肾炎的诊断意义

目的探讨抗核抗体(ANA)、免疫球蛋白和补体检测对狼疮性肾炎(LN)和不伴肾炎的系统性红斑狼疮(SLE)的诊断意义。方法对406例SLE患者和120例健康体检者采用间接免疫荧光法测定ANA,应用散射比浊法测定补体(C3、C4)及免疫球蛋白(IgG、IgA、IgM、IgE)。结果 406例SLE患者中,ANA阳性率94.49%,与对照组比较,差异有统计学意义(P<0.01)。SLE患者LN组与非LN组ANA核型均以核均质型和核颗粒型为主,LN组这2种核型占84.35%,非LN组占72.12%,两者差异有统计学意义(P<0.05);LN组胞浆颗粒型占11.31%,与非LN组的21.93%差异有统计学意义(P<0.05)。LN组与非LN组IgG、IgA、IgM、IgE水平均增高,而C3、C4水平均降低,与对照组比较差异有统计学意义(P<0.05);C3水平在LN组降低更明显,与非LN组比较差异有统计学意义(P=0.001)。结论 ANA、免疫球蛋白和补体的联合检测对提高SLE的临床诊断、预后判断、疗效观察等方面意义重大。