系统性红斑狼疮患者T淋巴细胞PTA1表达的研究

目的：研究血小板和T细胞活化抗原1（PTA1）在系统性红斑狼疮（SLE）外周血T淋巴细胞的表达及其与SLE活动相关性，探索活化T细胞在SLE发病中的作用。方法：应用双色直接荧光标记，流式细胞仪分析27例（其中活动期13例，非活动期14例）SLE患者和30名健康志愿者的CD3，CD4，CD8淋巴细胞，在植物血凝素（PHA）刺激下PTA1（CD226）表达，同时检测SLE患者抗dsDNA抗体，C3和C4补体，疾病活动度用SLEDAI记分，结果：SLE患者组CD3，CD4，CD8淋巴细胞上PTA1表达率均高于正常对照组，CD3上PTA1表达两组差异有显著性（P＜0．01），活动期SLE组CD3，CD4，CD8细胞上PTA1表达均高于正常对照组和非活动期SLE组（P＜0．01），而非活动期SLE组与正常对照组差异无显著性（P＞0．05），SLE患者CD3，CD8细胞PTA1表达与SLEDAI，抗dsDNA抗体之间呈正相关，与C3，C4补体水平呈负相关，CD8细胞PTA1表达与SLEDAI，抗dsDNA抗体，C3，C4补体水平呈直线相关（P＜0．05），结论：SLE患者存在T细胞亚群异常活化，活动期SLE淋巴细胞PTA1表达增高，SLE患者CD8细胞PTA1表达异常与SLEDAI，抗dsDNA抗体，C2和C4补体之间有明显相关，CD8细胞活化程度与SLE疾病程度有关，PTA1可能参与了SLE的免疫发病机制。     

系统性红斑狼疮患者抗淋巴细胞抗体变化与免疫功能失调

目的　探讨系统性红斑狼疮 (SLE)患者免疫功能失调机制。方法　微量淋巴细胞毒法。在狼疮活动期和缓解期分别查抗淋巴细胞抗体 (ALA)、淋巴细胞形态及计数。结果　 2 3例SLE患者活动期有 2 0例ALA阳性伴淋巴细胞肿胀变性、胞膜毛糙失去折光性 ,占 86 9% ,淋巴细胞计数为 (1 5± 0 6 )× 10 9/L而经激素治疗病情缓解后ALA阳性且淋巴细胞肿胀变性者仅 9例 ,占39 1% ,淋巴细胞计数上升为 (3 5± 1 0 )× 10 9/L。前后比较差异有显著性 (P <0 0 1)。结论　SLE患者活动期体内存在ALA并导致淋巴细胞变性 ,细胞数目减少 ,免疫功能失调。激素治疗可逆转上述病理变化。     

血清B淋巴细胞刺激因子在系统性红斑狼疮中的意义

目的 研究血清B淋巴细胞刺激因子（Blys）在系统性红斑狼疮（SEE）中的意义。方法 酶联免疫吸附试验（ELISA）法检测125例SLE患者血清Blys水平，并与狼疮活动程度和临床表现进行相关性分析。结果 125例SLE患者血清平均Blys水平显著高于正常对照组[（0．72±0．18）ng／ml vs（0．34±0．10）ng／ml，P=0．0031]；SLE患者血清平均Blys水平与SLE疾病活动指数（SLEDAI）积分呈一定程度相关性（r=0．73，P=0．018）；SLE患者血清Blys水平与抗dsDNA抗体滴度呈正相关，而与血清IgG、补体、蛋白尿、白细胞、血小板计数，以及皮疹、关节痛、口腔溃疡、脱发、发热等临床表现等均无显著相关性（P〉0．05）。结论 SLE患者血清Blys水平升高，与狼疮活动呈一定相关性，但不能准确反映病变部位及严重程度。     

抗核小体抗体、抗双链DNA抗体和抗超敏双链DNA抗体与系统性红斑狼疮的相关性研究

目的 通过检测系统性红斑狼疮(SLE)患者血清中抗核小体抗体(AnuA)、抗双链DNA (dsDNA)抗体和抗超敏双链DNA (dsDNA-NcX)抗体的水平,分析其在SLE患者中的敏感性、特异性及与其他实验室指标的相关性.方法 采用酶联免疫吸附试验(ELISA)法分别检测91例SLE患者、45例非SLE疾病对照和46例健康对照组血清中AnuA、抗dsDNA抗体和抗dsDNA-NcX抗体的水平,比较3种抗体对SLE诊断的敏感性和特异性,评价其与其他实验室指标的关系.结果 SLE患者AnuA、抗dsDNA抗体和抗dsDNA-NcX抗体的阳性率分别为49.45％、56.04％和61.54％;特异性分别为94.51％、94.51％和100.00％.AnuA、抗dsDNA抗体和抗dsDNA-NcX抗体均与SLEDAI评分呈正相关(r=0.50,P=0.00;r =0.49,P=0.00;r =0.42,P=0.00).ANA的滴度与AnuA的浓度呈正相关(r=0.30,P=0.00),与抗dsDNA抗体的滴度无相关性(r=0.19,P=0.08),与抗dsDNA-NcX抗体的浓度呈正相关(r=0.50,P=0.00).红细胞沉降率在抗dsDNA-NcX抗体、抗dsDNA抗体阴性和阳性组间比较差异无统计学意义(x2=0.76,P=0.38;x2=0.13,P=0.18),而在AnuA阴性与阳性组间比较差异有统计学意义(x2 =20.31,P=0.00).CRP及24小时尿蛋白定量在三者阴性与阳性组间比较差异无统计学意义.补体C3、C4在AnuA、抗dsDNA抗体和抗dsDNA-NcX抗体的阴性与阳性组间比较差异有统计学意义(x2=9.84,P=0.00;x2=16.53,P=0.00;x2 =10.33,P=0.00;x2 =11.61,P=0.00;x2 =12.69,P=0.00;x2=8.77,P=0.00).胱抑素在抗dsDNA抗体和AnuA的阴性与阳性组间比较差异无统计学意义,而在抗dsDNA-NcX抗体阴性与阳性组间比较差异有统计学意义(x2 =4.04,P=0.04).结论 抗dsDNA-NcX抗体可作为SLE的特异性抗体之一,其敏感性和特异性均高于抗dsDNA抗体和AnuA,三者均与SLE的疾病活动相关,联合检测有助于评估病情.     

自身抗体联合检测在系统性红斑狼疮诊断中的意义

目的研究抗细胞膜DNA(cmDNA)抗体、抗核小体抗体(AnuA)、抗脱氧核糖核蛋白(DNP)抗体及抗双链DNA(dsDNA)抗体等特异性抗体在系统性红斑狼疮(SLE)诊断中的意义,并与抗核抗体(ANA)的敏感性和特异性进行比较。了解特异性自身抗体联合检测在SLE的诊断及临床应用中的意义。方法测定了125例SLE及118例疾病对照组(包括原发性干燥综合征、多发肌炎、系统性硬化症、未分化结缔组织病、类风湿关节炎、骨关节炎、强直性脊柱炎及银屑病关节炎)患者血清中的自身抗体。利用间接免疫荧光法测定抗cmDNA抗体和ANA,酶联免疫吸附试验(ELISA)测定AnuA、乳凝法检测抗DNP抗体、金标法测定抗dsDNA抗体。结果AnuA、抗cmDNA抗体、抗DNP抗体、抗dsDNA抗体和ANA在SLE患者中的阳性率分别为68%、38.4%、51.2%、49.6%和95.2%,均明显高于疾病对照组(3.4%、4.2%、1.7%、0.8%和25.4%),差异有统计学意义(P<0.001)。ANA与AnuA的敏感性显著高于其他三种抗体,差异有统计学意义(P<0.05);AnuA、抗cmDNA抗体、抗DNP抗体、抗dsDNA抗体和ANA的特异性分别为95.8%、96.6%、98.3%、99.2%和74.6%;AnuA在抗DNP抗体、抗dsDNA抗体阴性的SLE中的阳性率明显高于其他抗体(P<0.05);自身抗体的联合检测可提高SLE诊断的敏感性,但特异性无明显改变。结论抗cmDNA抗     

系统性红斑狼疮患者T细胞上Fas受体分子与疾病活动指数的相关性研究

目的通过定量流式细胞术检测系统性红斑狼疮(SLE)患者T淋巴细胞表面Fas受体的分子数,探讨SLE的发病机制及与疾病活动指数的相关性.方法对36例活动期SLE患者和18名正常人群采集外周血,采用Fas定量流式细胞试剂盒荧光染色,通过流式细胞仪对T淋巴细胞上表达Fas受体分子数和凋亡率进行检测.结果活动期SLE患者T细胞表面表达Fas受体分子数和凋亡率较正常人群明显上调(P＜0.01).活动期SLE患者T细胞表面Fas受体分子数与T细胞凋亡率和SLEDAI之间,具有明显的正相关(P＜0.01),抗dsDNA抗体阳性患者组T细胞表面Fas受体分子数较抗dsDNA抗体阴性组明显增高,差异具有统计学意义(P＜0.05).结论SLE患者T淋巴细胞表面Fas受体分子数上调,由Fas介导的T淋巴细胞凋亡加快,刺激机体产生抗dsDNA等多种自身抗体,可能是引起SLE免疫功能紊乱的主要原因.SLE患者T淋巴细胞表面Fas受体分子数与SLE的活动性呈正相关,是一较好的评价SLE疾病活动性的指标.     

白芍总苷对系统性红斑狼疮患者周围血象变化的影响

为了解白芍总苷(TGP)对已有周围血白细胞减低的系统性红斑狼疮(SLE)患者的安全性与疗效,采用1∶1配比病例对照研究的方法。借助SPSS10.0软件包辅助统计与分析有关资料。结果观察组与对照组治疗前血红蛋白定量、周围血白细胞与淋巴细胞和血小板计数、血沉、血清补体C3水平、血清抗dsDNA的阳性率、系统性红斑狼疮活动指数(SLEDAI)以及初始平均每日应用泼尼松的剂量差异均无显著性(P0.05)。观察组用TGP治疗6周后血红蛋白定量、周围血白胞及淋巴细胞和血小板计数与对照组相比差异无显著性(P0.05)。但平均血沉水平、血清抗dsDNA的阳…     

骨桥蛋白在系统性红斑狼疮中的临床意义

目的 研究血清骨桥蛋白(OPN)与系统性红斑狼疮(SLE)临床表现、实验指标、疾病活动的相关性,探讨血清OPN在SLE中的意义及SLE的发病机制.方法 用酶联免疫吸附(ELISA)法检测68例SLE患者和20名正常对照组血清中OPN的水平,并监测SLE患者的临床表现及实验室指标,分析其与OPN的相关性.结果 68例SLE患者中79%血清OPN阳性,20名正常对照组0PN均阴性,SLE组患者血清OPN阳性率及OPN水平显著高于正常对照组(P＜0.01).血清OPN阳性组的SLE患者与OPN阴性组的SLE患者相比,在年龄、性别及病程上差异均无统计学意义(P＞O.05 .血清OPN阳性组SLE患者发热、脱发、白细胞降低、肝脏损害、补体C4降低、免疫球蛋白IgA增高、蛋白尿及抗dsDNA抗体阳性的发生率明显高于OPN阴性组(P＜0.05). SLE患者血清OPN阳性率明显高于抗dsDNA抗体、抗sm抗体的阳性率,差异具有统计学意义(P＜0.01). SLE患者血清OPN水平与SLE疾病活动指数(SLEDAI)积分呈明显的正相关(r=0.292;P＜0.05);SLE活动期患者血清OPN阳性率及水平明显高于SLE缓解期患者,两组比较差异具有统计学意义(P＜O.05).结论 SLE患者血清中OPN水平升高,与SLE疾病活动性密切相关,可作为SLE的病情活动指标.     

抗C1q抗体与系统性红斑狼疮疾病活动及狼疮肾炎的关系

目的研究抗C1q抗体与系统性红斑狼疮（systemic lupus erythematosus,SLE）疾病活动及肾损害的相关性。方法使用ELISA法测定93例初诊SLE患者和69例其他风湿性疾病患者及32名健康对照者血清中抗C1q抗体的浓度。同时记录SLE疾病活动指数（systemic lupus erythematosus Disease Activity Index,SLEDAI）、自身抗体和相关实验室指标。结果血清抗C1q抗体、抗dsDNA抗体、抗核小体抗体（anti-nucleosome antibodies,AnuA）,SLE组的阳性率分别为40.9%、62.4%和62.8%。血清抗C1q抗体阳性组患者的肾损害发生率（84.2%）明显高于阴性组（23.6%）。狼疮肾炎（lupus nephritis,LN）患者血清抗C1q抗体浓度（55.36±51.96）RU/ml及阳性率（71.1%）显著高于无肾炎表现的狼疮对照组（12.09±14.46）RU/ml,12.5%。无论患者有无肾损害,狼疮疾病活动患者抗C1q抗体浓度（62.46±50.29）RU/ml及阳性率（85.7%）显著高于疾病稳定的狼疮对照组（8.79±6.42）RU/ml,3.9%。而AnuA和抗dsDNA抗体只在伴有狼疮肾损害的疾病活动组即活动LN的阳性率（87.9%与50.0%）显著高于非活动LN对照组（66.7%与33.3%）。LN组抗C1q抗体、AnuA、抗dsDNA和抗Sm（Smith）抗体阳性率显著高于非LN组（71.1%与12.5%）,（77.8%与47.9%）,（57.8%与25.0%）,（31.1%与10.4%）。SLE疾病活动组抗C1q抗体、AnuA、抗dsDNA阳性率显著高于疾病稳定组（85.7%与3.9%）,（88.1%与41.2%）,（61.9%与17.6%）。活动LN抗C1q抗体、AnuA、抗dsDNA阳性率显著高于非活动LN（93.9%与8.3%）,（87.9%与50.0%）,（66.7%与33.3%）。活动LN组抗C1q抗体、AnuA、抗dsDNA及抗中性粒细胞胞质抗体（ANCA）阳性率显著高于其他SLE组（93.9%与13.1%）,（87.9%与48.3%）,（66.7%与26.7%）,（43.5%与8.5%）。结论血清抗C1q抗体能够反映SLE的疾病活动,并与SLE肾损害相关,是对活动性LN敏感的自身抗体。     

老年系统性红斑狼疮患者血清干扰素调节因子的检测及意义

目的探讨老年系统性红斑狼疮(SLE)患者血清干扰素调节因子4(IRF4)的水平及其与临床相关资料相关性。方法比较SLE组82例与正常组68例、活动性指数(SLEDAI)≥6与SLEDAI<6组、狼疮性肾炎(LN)组与非LN组间的IRF4水平差异,分析IRF4水平与SLE患者的炎性相关指标,包括SLEDAI、C-反应蛋白(CRP)、血细胞沉降率(ESR)、白细胞计数(WBC)、实验室指标包括ANA滴度、抗ds-DNA、抗SM抗体、抗SSA抗体、抗SSB抗体的相关性。结果 SLEDAI≥6的SLE患者的IRF4水平显著低于SLEDAI<6的患者(t=2.793,P<0.01);IRF4水平与SLEDAI、CRP、ESR、WBC均无明显相关性(P均>0.05);抗ds-DNA增加的SLE患者IRF4水平显著低于抗ds-DNA未增加患者(t=3.443,P<0.01);抗SSA抗体阳性患者的IRF4水平显著高于阴性患者(t=2.012,P=0.039)。结论 IRF4与老年患者SLE的发生有着密切的联系,且其水平与SLEDAI、抗ds-DNA增加与否、抗SSA抗体有着一定的关系。     

儿童系统性红斑狼疮神经精神症状的研究(二)

目的 探讨儿童神经精神性狼疮(NPSLE)的发病有无相关因素或预测指标.方法 回顾性分析1990-2006年诊断为SLE的62例中国儿童系统性红斑狼疮(SLE)患者的临床资料,分别比较有NPSLE组和无NPSLE组、早发NPSLE组和晚发NPSLE组、同组患者诊断NPSLE时和诊断SLE时等的临床资料.结果 有NPSLE组在诊断SLE时LN存在率、尿榆异常率和尿蛋白定量(24 h)值明显低于无NPSLE组;早发NPSLE组的平均年龄明显小于晚发NPSLE组,SLE疾病活动指数(SLEDAI)明显高于晚发NPSLE组;晚发NPSLE组在诊断NPSLE时和诊断SLE时临床表现和实验室检查等各项指标差异均无统计学意义.结论 在诊断SLE时存在狼疮肾炎(LN)的患者发生NPSLE概率降低;早发NPSLE大多年龄小,疾病活动性高;没有临床指标可以预测NPSLE的发生.     

Lymphopenia in systemic lupus erythematosus patients: Is it more than a laboratory finding?

Aim of the workTo investigate the relation between lymphopenia and clinical manifestations, laboratory findings, disease activity and damage index in systemic lupus erythematosus (SLE) patients.Patients and methods124 SLE patients were recruited from the Rheumatology Department in-patient and outpatient clinic, Faculty of medicine, Cairo University Hospitals. SLE disease activity index (SLEDAI) and the systemic lupus international collaborating clinics damage index (SLICC-DI) were assessed. Patients were divided into two groups according to presence or absence of lymphopenia (lymphocyte count ≤1500 cells/mm³⁾, and were compared as regards different disease parameters.ResultsThe 124 patients were 113 females (91.1%) and 11 males (8.9%). Their mean age was 28.54 ± 8.25 years and median disease duration was 48 (24–105) months. 86 (69.4%) had renal involvement, complement C3 was consumed in 84 (67.7%) and C4 in 44 (35.5%). All were receiving steroids and a high dose was more frequent in those with lymphopenia (17 (29.8%) compared to those without (in 9 (13.4%). Lymphopenia was found in 57 (46%) patients. Lymphopenic patients had higher frequency of renal involvement (p = 0.002) (OR = 3.37, CI = 1.5–7.8), complement consumption (p = 0.03), 24 h urinary proteins (p = 0.034), higher steroid doses (p = 0.012) and cyclophosphamide administration (p = 0.03). SLEDAI was severe in 29.8% of lymphopenic patients (p = 0.16), and SLICC-DI median was 2 in both groups (p = 0.4).ConclusionLymphopenia is a common finding in SLE patients and was significantly associated with lupus nephritis, complement consumption, higher steroid doses and cyclophosphamide administration. Lymphopenia might be a promising marker for renal involvement in SLE.     

抗N-甲基-D-天冬氨酸型受体亚型NR2a/2b抗体与神经精神性狼疮

目的 探讨抗N-甲基-D-天冬氨酸型(NMDA)受体亚型NR2a/2b抗体(抗NR2抗体)在神经精神性狼疮(NPSLE)患者免疫发病机制中的作用和意义.方法 采用酶联免疫吸附试验(ELISA)法检测59例NPSLE患者、54例无神经精神症状的系统性红斑狼疮(SLE)患者血清及20例NPSLE患者脑脊液中抗NR2抗体的水平,评价抗NR2抗体与NPSLE的临床表现、病情活动度评分(SLEDAI)、抗dsDNA抗体水平、抗核糖体P蛋白抗体、抗核抗体(ANA)、抗Sm抗体的相关性.结果 59例NPSLE患者血清中的抗NR2抗体水平显著高于54例非NPSLE患者.同时2组患者SLEDAI评分、抗Sm抗体阳性率、抗dsDNA抗体水平及抗核糖体P蛋白抗体水平差异均有统计学意义;59例NPSLE患者中,15例器质性脑病(认知功能障碍、记忆力减退)患者血清中的抗NR2抗体水平显著高于其他NPSLE患者;20例NPSLE患者脑脊液中仅有2例抗NR2抗体高滴度阳性,二者血清中的抗NR2抗体同样高滴度阳性,此2例患者均表现为认知功能障碍、记忆力减退,二者预后差.结论 检测SLE患者血清中的抗NMDA受体亚型NR2a/2b抗体对于NPSLE具有初步筛选作用;在表现为认知功能障碍、记忆力减退的NPSLE患者的血清及脑脊液中存在高滴度的抗NMDA受体亚型NR2a/2b抗体.     

Anti-tubulin-α-1C autoantibody in systemic lupus erythematosus: a novel indicator of disease activity and vasculitis manifestations

A variety of autoantibodies has been involved in the pathogenesis of systemic lupus erythematosus (SLE), some of which are well known and applied as disease biomarkers. This study aimed to determine the prevalence of a novel autoantibody, anti-tubulin-α-1C, in patients with SLE and investigate its clinical significance. Anti-tubulin-α-1C autoantibody levels were determined by enzyme-linked immunosorbent assay (ELISA) in 128 SLE patients, 38 primary Sjögren’s syndrome (pSS) patients, and 106 healthy controls (HCs).White blood cell (WBC) count, erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), IgM, IgG, C3, C4, RF, ANA, dsDNA, Sm, AnuA, aCL, anti-SSA, and anti-SSB were measured by standard laboratory techniques. SLE Disease Activity Index (SLEDAI) was evaluated accordingly. Anti-tubulin-α-1C antibody levels were significantly increased in SLE patients. Elevated anti-tubulin-α-1C were correlated with higher levels of SLEDAI, increased titers of anti-Sm antibody, and decreased titers of anti-dsDNA antibody and significantly associated with cutaneous and mucosal vasculitis and milder renal involvement. Anti-tubulin-α-1C may become a novel biomarker indicative of active vasculitis in SLE and could be applied in future clinical practice.     

神经相关性自身抗体检测在神经精神性狼疮中的意义

目的 探讨神经相关性自身抗体在系统性红斑狼疮(SLE)和神经精神性狼疮(NPSLE)患者血清或脑脊液中的敏感性和特异性,评价其在NPSLE诊断中的意义.方法 利用间接免疫荧光法分别以成神经瘤细胞株(SK-N-SH)、神经胶质瘤细胞株(U251)以及C57BL/10小鼠的大脑及脊髓切片为底物检测121例SLE患者、34例疾病对照组和34名健康对照组的血清及24例NPSLE患者和22例疾病对照组的脑脊液中抗神经元细胞抗体、抗神经胶质细胞抗体、抗脑抗体及抗脊髓抗体.结果 SLE患者血清中抗神经元细胞抗体、抗脑抗体及抗脊髓抗体的阳性率(17.4%、25.6%和29.8%)明显高于类风湿关节炎(RA)患者以及正常对照组(P＜0.05或P＜0.01),在SLE诊断中的特异性分别为98.5%、95.6%和100%;抗神经元细胞抗体、抗脑抗体及抗脊髓抗体均与SLE疾病活动性指数(SLEDAI)积分呈正相关,并与NPSLE患者的癫痫发作、头痛、急性意识障碍以及情绪失调、焦虑等症状的发生有关;NPSLE患者血清的抗神经元细胞抗体、抗脑抗体和抗脊髓抗体的阳性率(27.8%、38.9%和61.1%)明显高于无中枢神经系统受累的SEE患者(P＜0.05或P＜0.01);NPSLE患者的脑脊液中抗脑抗体、抗脊髓抗体的阳性率均为29.2%,与疾病对照组比较,差异具有统计学意义(P＜0.05或P＜0.01);在NPSLE诊断中的特异性分别为100%和95.5%.结论 抗神经元细胞抗体、抗脑抗体及抗脊髓抗体对SLE诊断的特异性较高,但敏感性偏低;3种抗体均与SLE疾病活动性相关;血清中抗神经元细胞抗体、抗脑抗体及抗脊髓抗体是NPSLE诊断的较特异性抗体,抗脊髓抗体可能是诊断NPSLE较敏感的指标之一;脑脊液中的抗脑抗体、抗脊髓抗体对NPSLE的诊断更具有特异性.     

Elevated levels of soluble fractalkine in active systemic lupus erythematosus: potential involvement in neuropsychiatric manifestations

OBJECTIVE: To determine levels of the soluble form of the chemokine fractalkine (sFkn) and its receptor, CX(3)CR1, in patients with systemic lupus erythematosus (SLE) with neuropsychiatric involvement (NPSLE) and in SLE patients without neuropsychiatric involvement, and to assess their relationship with disease activity and organ damage. METHODS: Levels of sFkn in serum and cerebrospinal fluid (CSF) were measured by enzyme-linked immunosorbent assay. Expression of Fkn and CX(3)CR1 was quantified using real-time polymerase chain reaction. Surface expression of CX(3)CR1 on peripheral blood mononuclear cells (PBMCs) was determined by flow cytometry. Disease activity and organ damage were assessed using the SLE Disease Activity Index (SLEDAI) and the Systemic Lupus International Collaborating Clinics/American College of Rheumatology (SLICC/ACR) Damage Index, respectively. RESULTS: Serum sFkn levels were significantly higher in patients with SLE than in patients with rheumatoid arthritis (RA) or healthy controls. In addition, significant correlations between serum sFkn levels and the SLEDAI, the SLICC/ACR Damage Index, anti-double-stranded DNA and anti-Sm antibody titers, immune complex levels (C1q), and serum complement levels (CH50) were observed. Expression of CX(3)CR1 was significantly greater in PBMCs from patients with active SLE than in those from RA patients or healthy controls. Levels of sFkn were also significantly higher in CSF from untreated patients with newly diagnosed NPSLE than in SLE patients without neuropsychiatric involvement; treatment reduced both serum and CSF levels of sFkn in patients with SLE. CONCLUSION: Soluble Fkn and CX(3)CR1 may play key roles in the pathogenesis of SLE, including the neuropsychiatric involvement. Soluble Fkn is also a serologic marker of disease activity and organ damage in patients with SLE, and its measurement in CSF may be useful for the diagnosis of NPSLE and followup of patients with NPSLE.     

Clinical features and outcome of neuropsychiatric lupus in Chinese: analysis of 240 hospitalized patients

Neuropsychiatric (NP) events are severe manifestations of systemic lupus erythematosus (SLE) and relate to poor outcome. The aims of this study are to investigate the NP manifestations of SLE and to identify the predictive factors for clinical outcome. There was a retrospective review of 240 hospital patients with primary NP events of SLE (NPSLE) from 1990 to 2004. Neuropsychiatric manifestations, SLE disease activity index (SLEDAI) score, System lupus International Collaborating Clinic/American College of Rheumatology Damage Index (SLICC/ACR-DI) score, magnetic resonance imaging (MRI) findings, treatment and mortality rate were included for analysis. From this group of patients, 15 NP syndromes were identified. The most frequent manifestation was headache, followed by seizure. The mean SLEDAI and SLICC/ACR-DI scores were 19.9 +/- 6.9 and 3.5 +/- 1.6, respectively. Abnormal MRI features were found in 67% (61/91) patients. At least one intrathecal (IT) injection of methotrexate (MTX) plus dexamethasone (DXM) was administered to 109 (45.4%) patients. High dose (1 g) intravenous methylprednisolone pulse therapy (IVMP) was administered to 167 (69.5%) patients. Multifactor analysis revealed that high SLICC/ACR-DI scores and sets of concurrent NP symptoms were independently associated with poor outcome, whereas pulse IVMP and IT injection of MTX plus DXM were protective factors against poor outcome. From our data, NPSLE is heterogeneous and is usually associated with high disease activity and organ damage scores. High SLICC/ACR-DI score and having more than two sets of NP symptoms are the predictors for poor outcome, whereas pulse IVMP and IT injection of MTX plus DXM can improve the prognosis.     

系统性红斑狼疮患者干扰素诱导基因的表达及其与疾病活动性的相关性研究

目的 探讨系统性红斑狼疮(SLE)患者外周血单个核细胞(PBMCs)中5个干扰素(IFN)诱导基因(IFIT1、IFIT4、OAS1、OASL、ISG15)的表达及其与SLE疾病活动度的相关性.方法 运用SYBRgreen dye I实时定量聚合酶链反应(RT-PCR)方法检测76例SLE患者与54名健康对照人的IFIT1、IFIT4、OASL、OASL及ISG15 mRNA表达水平(以-AACt值表示),并且与红细胞沉降率(ESR)、C反应蛋白(CRP)、补体C3、C4、抗核抗体(ANA)及抗dsDNA抗体等指标比较,分析IFIT1、IFIT4、OAS1、OASL、ISG15 mRNA表达水平以及上述常规检测指标与SLE疾病活动指数(SLEDAI)积分之间的相关性.结果 ①SLE组与正常对照组相比,IFIT1、IFIT4、OAS1、OASL及ISG15 mRNA表达显著增高(P＜0.01);SLE活动组与SLE缓解组比较,IFIT1、IFIT4、OAS1、OASL及ISG15 mRNA表达增高(P＜0.05);SLE组IFIT1、IFIT4、OAS1、OASL及ISG15 mRNA表达水平之间呈正相关(r＞0.5,P＜0.05).②SLE组IFIT1、IFIT4、OAS1、OASL及ISG15 mRNA表达水平均与SLEDAI积分呈显著正相关(r＞0.5,P＜0.05).③ESR、CRP、补体C3、C4、ANA与IFIT1、IFIT4、OAS1、OASL、ISG15 mRNA表达水平及SLEDAI积分均无相关性,抗dsDNA抗体与IFIT1、IFIT4、OAS1、OASL、ISG15 mRNA表达水平及SLEDAI积分呈正相关(r＞0.5,P＜0.05).结论 SLE患者IFIT1、IFIT4、OAS1、OASL及ISG15的表达水平在SLE患者中显著增高,并且与SLEDAI积分呈显著正相关,对SLE患者病情活动度和病情严重度的判断均有较大价值,抑制这5个基因的表达可能为SLE的治疗提供新的靶点.     

Disease activity, damage and survival in Mexican patients with acute severe systemic lupus erythematosus

Systemic lupus erythematosus (SLE) is a clinical syndrome of varying severity. Although the survival and prognosis of SLE have steadily improved, there is a group of patients who present an acute fatal outcome despite aggressive therapy. We designed this study to evaluate the factors associated with mortality in patients with acute severe SLE. During 2004-06, 41 Mexican SLE patients that could not be managed in the out-patient clinic and with acute severe major organ system involvement [nephritis, severe thrombocytopenia (platelet count below 20 000 per microL) acute neuropsychiatric pulmonary, gastrointestinal or cardiac disease and generalized vasculitis] were studied. During the first admission, disease activity (SLE Disease Activity Index (SLEDAI), SLE Activity Measured), damage [SLE International Collaborating Clinics (SLICC)], and therapy were assessed. Survival using Kaplan-Meier curves, odd ratios with 95% confidence interval and logistic regression analysis were used to determine risk factors for mortality. Ninety percent were female with a mean age of 29 +/- 19 years and mean disease duration of 21 +/- 9 months. The principal causes of first admission were renal (27%), SNC (22%) and cardiopulmonary (15%). After a mean follow-up of 9.7 +/- 6 months, 16 (39%) patients died. Deceased patients had significantly higher SLEDAI (P = 0.004), and SLICC (P = 0.03) scores. The manifestations associated with mortality were renal disease activity (odds ratio, OR 4.6, confidence interval, CI 95% 1.0-20.6), infections (OR 3.2 CI 95% 2.0-5.3) and thrombocytopenia (OR 4.0, CI 95% 1.0-15.9). The survival at 9.7 months was 72, 62 and 50% in patients with an SLEDAI score of 3-10, 11-20 and > or =21, respectively. The SLEDAI score, the presence of damage and infection were associated with death in patients with acute severe SLE.     

Neuropsychiatric systemic lupus erythematosus presenting as amyotrophic lateral sclerosis

A 61-year-old woman with a history of photosensitive dermatitis and recurrent mouth ulcers presented with progressive weakness typical of amyotrophic lateral sclerosis (ALS), and subsequently underwent extensive neurologic and rheumatologic testing. We investigated whether ALS-like motor neuron disease associated with a positive antinuclear antibody (ANA) is really ALS or rather neuropsychiatric systemic lupus erythematosus (NPSLE). On neurologic evaluation, she had prominent bulbar involvement with dysarthria and dysphagia associated with profound lingual fasciculations and a denervating pattern on electromyogram. MRI showed no evidence of cerebral ischemia. Laboratory studies revealed a positive ANA (1:2560 titer), positive antiphospholipid antibodies (GPL and MPL), circulating lupus anticoagulant, and depressed C3 and C4. Repeat MRI studies at 4 and 11 mo revealed an evolving infarct in the paramedian pons consistent with the presence of NPSLE. Therapy was initiated with corticosteroids and intravenous cyclophosphamide, and the neurologic condition did not improve, but also did not progress inexorably as would be expected with ALS. NPSLE, presumably through the mechanism of ischemic vasculopathy, may present as motor neuron disease clinically indistinguishable from ALS.