狼疮肾炎患者左心结构与功能改变的多因素分析

目的 探讨狼疮肾炎患者左心结构和功能的改变与年龄、病程、血压、血红蛋白、肌酐、肾小球滤过率等因素的相关性.方法 将132例系统性红斑狼疮(SLE)患者分为狼疮肾炎组与非狼疮肾炎组,对2组临床资料进行t检验x2检验、Pearson相关件分析及多因素回归分析.结果 与非狼疮肾炎患者的向心性肥厚发生率(4/67,6％)相比,狼疮肾炎患者的向心性肥厚发生率(14/65,22％)明显增高(x2=6.790,p＜0.05).狼疮肾炎患者左心室收缩末期内径与尿酸呈正相关(B=0.014,P＜0.01),左心室心肌质量指数与尿素氮呈正相关(B=2.977,P＜0.01),室间隔厚度与收缩压呈正相关(B=0.022,P＜0.01).结论 狼疮肾炎患者向心性肥厚较非狼疮肾炎者发生率高,考虑与SLE累及肾脏进而引起高血压、尿酸与尿素氮升高等有关,这些因素可进一步加重心脏损害.     

系统性红斑狼疮患者肾脏改变与临床表现相关性的研究

目的研究系统性红斑狼疮(SLE)患者临床表现、部分实验室检测结果与肾脏病理损害类型之间的关系.方法将96例作肾脏活组织检查的SLE患者依据有无狼疮肾炎(LN)的临床表现分为两组,即有肾炎症状狼疮肾炎组(OLN)(48例)和无肾炎症状狼疮肾炎组(SLN)(48例),分析肾脏穿刺结果部分和部分实验室检测结果.结果两组患者均有肾脏活检证实的肾小球损害(100%),但SLN组病理损害类型以Ⅰ、Ⅱ型为主(39/48,81%),尤以Ⅱ型患者为多(35/48,73%)(P＜0.01),OLN组患者的病理损害类型以Ⅳ型为主(32/48,67%),Ⅲ型以上者为88%(42/48)(P＜0.01);且SLN组肾脏损害活动性和迁延性评分(活动指数、慢性化指数)显著低于OLN组(P均＜0.01).部分实验室检查结果显示,两组患者在性别、年龄、SLEDAI评分和自身抗体水平方面及补体C4差异无显著性(P＞0.05).OLN组患者循环免疫复合物(CIC)、血沉升高及补体C3降低较SLN组更为显著(P均＜0.01).肾功能放射免疫5项检测显示,与SLN组相比,除尿β2-微球蛋白(β2-MG)外,OLN组患者血β2-MG、尿白蛋白(Alb)、尿IgG、尿α2-MG等4项检测均显著增高(P均＜0.01),且SLN组5项均正常者为38%(18/48),4项以上不正常者仅为12%(6/48),而OLN组患者4项以上不正常者为71%(20/28),无一例5项均正常者(0/28)(P均＜0.01).两组一般临床资料分析则显示,前者的病程明显长于后者(P＜0.01).结论SLE患者的肾脏损害可始于疾病的早期.随着病程的延长,患者肾外表现逐渐增多,肾脏损害程度也渐趋严重.因此,对于SLE患者或疑似患者,即使无狼疮肾炎临床表现,亦应积极行肾脏活检穿刺术,以从病理学角度明确SLE及狼疮肾炎的诊断.     

我国类风湿关节炎患者羟氯喹使用的现状调查

目的 了解我国类风湿关节炎(RA)患者羟氯喹使用的现状及特点.方法 现场调查全国20家大型医院风湿科就诊的858例RA患者,记录患者的一般人口学特征、临床病情活动度、羟氯喹用药情况以及不良反应等.采用SPSS 20.0软件根据不同数据特点进行Mann-Whitney U检验、x2检验及回归分析等.结果 ①羟氯喹在我国RA患者中使用方案较规范,使用率为19.5％(167/858),64.7％(108/167)达到足疗程,77.8％(130/167)达到推荐剂量,94.6％(158/167)为联合用药,最常用的联合药物为甲氨蝶呤和来氟米特.②足疗程使用羟氯喹的RA患者多项疾病活动指标得到明显改善,包括晨僵时间(U=1 670.5,P＜0.05)、压痛关节数(U=1 380.5,P＜0.01)及肿胀关节数(U=67 974.5,P＜0.01)、患者视觉模拟评分(VAS)疼痛评分(U=2 086.0,P=0.01)、患者VAS疾病活动评分(U=2 181.5,P＜0.05)、医生VAS疾病活动评分(U=2 086.5,P＜0.05)、28个关节疾病活动指数(DAS28)评分(U=827.5,P＜0.01)、斯坦福健康评定量表(HAQ)评分(U=1 855.5,P＜0.01)、红细胞沉降率(U=1 231.0,P＜0.05).③羟氯喹总体不良反应发生率为5.4％(9/167),最常见眼部受累(包括眼底黄斑变性、眼前闪光、眼异物感)及过敏反应、发生率分别为1.8％(3/167)及1.2％(2/167).结论 在我国大型医院就诊的RA患者中有19.5％使用羟氯喹,其中94.6％为联合用药.羟氯喹足疗程使用疗效肯定,总体不良反应发生率低,无严重不良反应.     

中国狼疮肾炎患者的临床特征及治疗选择：基于CSTAR队列的研究

目的分析总结狼疮肾炎(lupus nephritis, LN)患者的临床特征及初始治疗选择,提高对中国LN患者的认识。方法基于中国系统性红斑狼疮(systemic lupus erythematosus, SLE)研究协作组(Chinese SLE Treatment and Research group, CSTAR)建立的SLE起始队列,分析2009年2月至2021年3月登记注册的确诊时间在3个月内的8 713例SLE患者及其中LN患者的基线人口学特征、临床表现、实验室检查以及治疗选择。结果在初始诊断的SLE患者中,LN发生率为33.3%(2 900/8 713)。LN患者肾脏病理活检以Ⅳ型LN最多见(35.1%)。LN组患者男性比例更高(12.0%vs. 7.7%,P0.001),SLEDAI评分更高(11.7±8.5 vs.6.6±6.2,P0.001),SLICC/ACR脏器损伤评分≥1分的比例更高(34.0%vs. 16.6%,P0.001)。单因素及多因素分析结果显示血小板减少(23.2%vs. 16.6%,P0.001)、浆膜炎(19.2%vs. 8.4%,P0.001)、神经精神狼疮(7.6%vs. 5.5%,P=0.001)、肌炎(3.3%vs. 2.1%,P=0.006)、低补体血症(66.0%vs. 53.1%,P0.001)、抗dsDNA阳性(54.4%vs. 42.4%,P0.001)与LN相关,而LN患者关节炎发生比例降低(26.8%vs. 30.4%,P0.001)。与非LN组相比,LN组患者初始治疗方案中糖皮质激素(90.7%vs. 84.1%,P0.001)及激素冲击治疗(9.1%vs. 3.4%,P0.001)、环磷酰胺(27.0%vs. 11.7%,P0.001)、霉酚酸酯(21.6%vs. 10.1%,P0.001)、他克莫司(4.8%vs. 2.3%,P0.001)应用比例显著增高,而羟氯喹(67.4%vs. 73.1%,P0.001)、环孢素(4.3%vs. 5.9%,P=0.002)应用比例显著降低。结论 SLE中LN组较非LN组患者男性比例更高,病情活动度高,脏器损伤重。LN与浆膜炎、血小板减少等提示SLE病情活动的临床表现及实验室指标相关,初始治疗更积极。     

老年狼疮肾炎发病机制探讨

<正>狼疮肾炎(LN)是系统性红斑狼疮(SLE)的肾脏损害,约50%以上SLE患者有肾损害的临床表现,肾活检显示肾脏受累几乎为100%。肾衰竭是SLE患者死亡的常见原因。目前针对LN治疗以激素联合免疫抑制剂为主,在治疗期间老年患者更易继发感染〔1〕。SLE患者体内出现多种自身抗体。这些抗体主要与核抗原结合,自身抗体与抗原结合形成免疫复合物后沉积于肾脏,逐步发展为LN。突破免疫耐受是SLE及LN发生的     

系统性红斑狼疮足细胞病七例临床特征分析

目的 回顾性分析狼疮足细胞病(LP)患者的临床及实验室特征、治疗反应及预后.方法 选取2011年1月至2019年5月无锡市人民医院住院期间行肾活检证实的LP病例7例,回顾性分析其临床及实验室特征、治疗反应及预后.结果 7例LP中6例为女性,平均发病年龄(33±12)岁,SLE平均病程(69±64)个月,其中3例为初发初诊,6例为初次诊断狼疮肾损害.6例患者表现为肾病综合征(NS);2例表现为急性肾功能不全(ARF).LP患者肾脏表现与SLE疾病活动度不平行,自身抗体阳性率、低补体发生率均较低.经激素、免疫抑制剂治疗后6例患者肾脏病变缓解;随访过程中,2例患者出现肾脏复发.结论 LP以生育期女性好发,NS或伴AKI是其主要临床表现,肾脏表现与肾外表现不平行,激素、免疫抑制剂治疗敏感,部分患者治疗后可出现复发.     

糖皮质激素对系统性红斑狼疮CD4+Foxp3+T细胞水平的影响

目的 探讨糖皮质激素治疗对系统性红斑狼疮(SLE)患者外周血CD4+ Foxp3+ T细胞水平的影响以及CD4+Foxp3+T细胞与SLE疾病活动的相关性.方法 采用流式细胞术检测26例SLE患者和5名正常人外周血CD4+Foxp3+T细胞百分率,Spearman相关分析法分析CD4+Foxp3+T细胞与SLE疾病活动指标及糖皮质激素的相关性.结果 活动期SLE患者外周血CD4+Foxp3+T细胞百分率(2.4±1.6)%低于正常人(3.3±0.8)%,但差异无统计学意义;非活动期SLE患者外周血CD4+Foxp3+T细胞百分率(3.3±0.7)%与正常人比较差异无统计学意义.活动期SLE患者经糖皮质激素治疗后外周血CD4+Foxp3+T细胞(6.1±3.5)%较治疗前(3.9±2.4)%升高(P＜0.05).SLE患者外周血CD4+Foxp3+T细胞水平与年龄、病程、红细胞沉降率、尿蛋白定量(24 h)、血清补体C3浓度、血清抗双链DNA(anti-dsDNA)抗体水平及SLE疾病活动指数(SLEDAI)无相关性,但与糖皮质激素每日用量呈显著正相关(r=0.51,P＜0.05).结论 SLE患者外周血CD4+Foxp3+T细胞水平不能作为狼疮活动指标.糖皮质激素治疗可上调SLE患者外周血CD4+Foxp3+T细胞水平,CD4+Foxp3+T细胞水平上调可能是糖皮质激素治疗SLE有效的机制之一.     

狼疮肾炎分型再评价与治疗进展

系统性红斑狼疮(systemic lupus erythematosus,SLE)是一种多系统损害的自身免疫性疾病,肾脏损害最常见.SLE损害肾脏,可导致终末期肾功能衰竭,肾功能衰竭是SLE死亡的主要原因之一.因此,为了改善SLE预后,临床上一方面研究免疫抑制剂的合理使用,另一方面不断更新狼疮肾炎(lupusnephritis,LN)的病理分型,使之更好地指导治疗和判断预后.本文对2004年公布的LN分型再评价及治疗进展作一综述.     

51例系统性红斑狼疮患者妊娠结局

目的研究系统性红斑狼疮(systemic lupus erythematosus,SLE)患者妊娠不良结局的发生率及引起不良结局的相关因素。方法回顾性分析51例在本院风湿免疫科规律随访并最终在本院产科分娩的SLE患者的病历资料,总结患者的妊娠结局、新生儿情况以及妊娠期间服用药物、分娩前SLE疾病活动度评分(systemic lupus erythomatosus disease activity index,SLEDAI)。比较不同疾病活动度组患者在药物治疗、不良妊娠结局发生率方面的不同,并比较不良妊娠结局组(妊娠胎儿丢失、新生儿死亡)与活产组患者药物治疗及疾病活动情况。结果 51例SLE患者的平均年龄(28.0±3.9)岁,平均病程(4.7±4.5)年,妊娠期间26例(51.0%)应用羟氯喹治疗,31例(60.8%)应用糖皮质激素治疗,平均剂量为泼尼松5 mgd(0~30 mgd)。分娩(或引产)前51例患者的SLEDAI为(11.8±10.3)分,其中SLE中-重度活动组患者20例(39.2%),8例(15.7%)引产;病情缓解-轻度活动组患者31例,无1例引产。共出生新生儿45例(其中2对双胎),新生儿死亡2例(4.4%);活产43例新生儿中2例(4.7%)发生新生儿狼疮。与疾病缓解-轻度活动组患者相比,中-重度活动组患者的平均住院时间[(25.5±14.0)vs.(13.5±8.5)]、胎儿丢失率[10(50.0%)vs.0(0.0)]及早产率[9(63.6%)vs.9(21.4%)]均显著高于前者,差异有统计学意义(均P0.05);而应用糖皮质激素比例[8(40.0%)vs.23(60.7%)]及用药剂量[0(0,30)mg vs.6(0,20)mg]更低,但差异无统计学意义(P0.05)。不良妊娠结局(胎儿丢失及新生儿死亡)组分娩(或引产)前SLEDAI明显高于活产组[(21.3±8.9)vs.(7.1±7.9),P0.01],而妊娠期糖皮质激素应用比例少于活产组(30.0%vs.68.3%,P=0.032),但两组在羟氯喹应用剂量上无明显差别[0(0,20)mg vs.6(0,30)mg,P=0.096]。结论分娩前SLE活动度与妊娠不良结局(如胎儿丢失、早产及新生儿死亡)有关,疾病活动度高是发生妊娠不良结局的危险因素。     

系统性红斑狼疮患者血清卵泡抑素样蛋白1表达水平及其临床意义

目的 检测系统性红斑狼疮(SLE)患者血清中卵泡抑素样蛋白1(FSTL1)的水平及FSTL1在狼疮肾炎肾脏组织中的表达,探讨其在SLE疾病中的临床意义.方法 酶联免疫吸附试验(ELISA)法检测54例SLE患者及27名健康对照组血清FSTL1水平.免疫组织化学染色检测狼疮肾炎患者肾脏组织及健康者肾脏组织中FSTL1的表达情况.采用Mann-WhitneyU/检验、t检验、X2检验及Pearson检验进行统计分析.结果 SLE组FSTL1血清水平(26±21) μg/L显著高于健康对照组(12±14) μg/L(P＜0.01);SLE高血压组明显高于非高血压组(P＜0.01);SLE病程≥5年组与病程＜5年组之间差异有统计学意义(P＜0.01);血清FSTL1表达水平与SLE疾病活动指数(SLEDAI)评分(r=0.319,P=0.022)、年龄(r=0.700,P＜0.01)、病程(r=0.513,P＜0.01)、补体C4 (r=0.443,P=0.004)、总胆固醇(r=0.460,P=0.001)呈正相关;与血小板计数(r=-0.422,P=0.001)、抗双链DNA(dsDNA)抗体水平(r=--0.276,P=0.046)呈负相关.FSTL1主要表达在肾小管上皮细胞胞质内.结论 FSTL1在SLE患者血清中明显升高,且与疾病活动性有关.提示FSTL1在SLE发病机制中起一定作用.     

妊娠合并系统性红斑狼疮94例临床分析

目的 寻找妊娠合并系统性红斑狼疮(SLE)患者妊娠及产后不良母婴预后的因素.方法 回顾性分析北京协和医院妇产科收治的妊娠合并SLE患者的临床资料,根据SLE活动与否,将患者分为SLE不活动组和SLE活动组.用logistic回归分析影响不良母婴结局的危险因素.结果 妊娠合并SLE者97例,其中3例失访,94例SLE患者共96例次妊娠,96例次妊娠中SLE不活动组36例次,SLE活动组60例次.96例次妊娠中18例次为治疗性引产或人工流产,胎儿丢失7例次,活产71例次,3例新生儿死亡.SLE活动组早产、小于胎龄JD(SGA)、窒息发生率高于SLE不活动组(P＜0.05).logistic回归分析显示,胎儿不良结局与子痫前期/子痫、低血小板血症、SLE活动等因素有关(13值分别为2.463、2.228、2.769,P＜0.05).96例次妊娠中56例次孕前SLE稳定,其中22例次(39.3%)在妊娠期和产后发生SLE活动.共有24例子痫前期,2例子痫.合并狼疮肾炎(LN)者52例次,孕前LN控制稳定者有25例次(48.1%,25/52),其中稳定1年以上者22例次,妊娠期有12例次发生LN活动;而LN稳定短于1年者3例,妊娠期全部发生LN活动.4例产妇死亡,均发生于产后.logistic回归分析显示,子痫前期/子痫与LN活动呈正相关(β值2.658,P＜0.05),而SLE活动与孕前尿蛋白呈正相关(13值3.263,P＜0.05).结论 SLE患者妊娠后胎儿丢失、早产、SGA、新生儿窒息的发生率在SLE活动时显著增加.约1/3的SLE患者在妊娠后出现SLE活动,LN活动时子痫前期、子痫发生率显著升高.     

系统性红斑狼疮患者发病及就医行为的现况调查

目的 回顾性分析系统性红斑狼疮(SLE)的发病形式、临床特点以及患者发病后的就诊情况.方法 采用流行病学现况调查的研究方法,随机调查了300例SLE患者,了解其自发病以来的临床表现以及发病后的就诊情况,并采用SPSS 13.0统计软件包进行统计学分析.结果 ①在300例SLE患者中,男女之比为1:13.②首发临床表现以关节痛/炎最多,其次为皮疹、发热,分别占46.3%、34.0%、32.7%.与女性患者相比,男性更容易于发病时即出现肾脏损害.60.9%的患者于发病起1年内出现肾脏损害,提示自SLE发病起1年为肾脏损害的高发期.③患者首次就诊选择风湿免疫科者较少,仅占35.3%(106/300),但风湿免疫科的确诊比例最高,为99.1%(105/106).从出现临床症状到患者就诊的中位时间为半个月,从患者出现症状到确诊SLE的中位时间为3个月,有23.7%的患者需要1年以上方能确诊.结论 关节痛/炎、皮疹、发热为SLE最常见的首发临床表现.与女性患者相比,男性患者更容易出现肾脏损害.发病后不能正确选择科室就诊、诊断延误的SLE患者仍占相当大比例.     

Clinical characteristics of lupus myocarditis in Korea

Clinically important myocarditis is an unusual feature in systemic lupus erythematosus (SLE). We describe the clinical characteristics, management and outcomes of five SLE patients who developed severe left ventricular dysfunction. Four patients were female with mean age of 36.4 years. Three patients had both lupus myocarditis and lupus nephritis. Four patients had raised anti-dsDNA antibody titer and low complement level and two patients had positive IgG anticardiolipin antibody. Three patients were treated by high-dose corticosteroids, one patient by intravenous pulse methylprednisolone, and one patient by intravenous immunoglobulin and pulse cyclophosphamide with high dose corticosteroids. Left ventricular function improved markedly in four patients and all of them had no recurrence of lupus myocarditis up to follow-up of 33 months. However, one patient, who showed no improvement of left ventricular function, was expired due to sudden cardiac arrest. Lupus myocarditis should be treated by immunosuppressive therapy with high-dose corticosteroids and mostly the prognosis might be good with early treatment.     

The role of antimalarial agents in the treatment of SLE and lupus nephritis

Systemic lupus erythematosus (SLE) is a multisystem autoimmune disease that affects various organs. Lupus nephritis is one of the most common, and most important, serious manifestations of SLE. Antimalarial agents are part of the immunomodulatory regimen used to treat patients with SLE; however, their role in the treatment of patients with lupus nephritis in particular is less well recognized, especially by nephrologists. Not all antimalarial agents have been used in the treatment of lupus; this Review will focus on studies using chloroquine and hydroxychloroquine. In addition, this Review will briefly describe the history of antimalarial drug use in patients with SLE, the theorized mechanisms of action of the agents chloroquine and hydroxychloroquine, their efficacy in patients with SLE and those with lupus nephritis, their use in pregnancy, and potential adverse effects. The Review will also cover the latest recommendations regarding monitoring for hydroxychloroquine-associated or chloroquine-associated retinopathy. Overall, antimalarial drugs have numerous beneficial effects in patients with SLE and lupus nephritis, and have a good safety profile.     

Clinical approach to lupus nephritis: Recent advances

Kidney involvement is common in systemic lupus erythematosus (SLE). Its clinical presentations are highly variable, ranging from mild asymptomatic proteinuria and/or hematuria to rapidly progressive uremia. Histological evidence of lupus nephritis is present in most patients with SLE, even when they do not yet have clinical manifestations. Current classification ISN/RPS 2003 (International Society of Nephrology/Renal Pathology Society) of lupus nephritis was promoted by a widely perceived need to re-examine existing classification, provide clearer distinctions between the histological classes, and improve diagnostic reproducibility and interobserver agreement. Lupus nephritis is a serious disease whose prognosis can usually be improved dramatically by treatment, but treatment is potentially toxic, prolonged, and complex. Current treatment regimens combine corticosteroids with cyclophosphamide, azathioprine or ciclosporin; mycophenolate mofetil has received much recent attention as a potentially immune suppressive agent and less aggressive immunosuppressive regimens can be prescribed. SLE patients should be regular followed to detect early kidney involvement.     

Primary antiphospholipid syndrome as the forerunner of systemic lupus erythematosus

The objective of this study was to analyse whether primary antiphospholipid syndrome (PAPS) may precede and modify the characteristics of systemic lupus erythematosus (SLE). Out of the total 362 SLE patients in our service, 223 patients had antiphospholipid antibodies (aPL), of whom 110 met the criteria of antiphospholipid syndrome. In 26 cases (7.2%) PAPS appeared 5.5 years before the onset of lupus (PAPS+SLE Group). Their clinical findings were compared to lupus patients without (SLE only Group, n = 26) and with secondary APS (SLE+SAPS Group, n = 26). The prevalence of deep venous thrombosis, stroke/TIA, recurrent fetal loss, coronary heart disease and myocardial infarction was significantly higher in PAPS+SLE Group as compared to SLE only Group. The difference in prevalence of fetal loss (P = 0.014) between PAPS+SLE and SLE+SAPS Groups was also recorded. On comparison to PAPS+SLE Group, patients without APS (SLE only Group) were younger at onset of lupus, with more frequent flares and a higher prevalence of WHO type III/IV nephritis (P = 0.007), requiring higher doses of cyclophosphamide and corticosteroids. Lupus started in the form of PAPS in 7.2% of our SLE patients, who presented with more thrombotic and less inflammatory complications than in SLE patients without a prior or with a following secondary APS. Considering the long latency between the two diseases, PAPS may be a forerunner of lupus, but it may also coexist with SLE as an independent autoimmune disorder.     

Inflammatory bowel disease and lupus: A systematic review of the literature

Coexistence of systemic lupus erythematosus (SLE) should be considered in patients with inflammatory bowel disease (IBD) and complex extraintestinal manifestations and the diagnosis of IBD could be established either before or after the diagnosis of SLE. Differential diagnosis of concomitant SLE and IBD is difficult and should always exclude infectious conditions, lupus-like reactions, visceral vasculitis and drug-induced lupus.The underlying mechanism by which 5-ASA/sulphasalazine induces SLE or lupus-like syndromes is not clear and high awareness for possible predictive factors is demanded for early prevention.In most cases the symptoms from drug-induced lupus have been reversible after the discontinuation of the drug and response to steroids is favorable. Treatment of patients co-diagnosed with SLE and IBD may include corticosteroids, immunosupressants and hydroxychloroquine.In severe lupus and IBD patients cyclophosphamide pulse may be of benefit while infliximab may be beneficiary in patients with lupus nephritis. However, the role TNFalpha plays in humans with SLE and IBD is controversial and data on the likely effects of blocking TNFalpha on anti-DNA autoantibody production is always of interest.     

Mycophenolate mofetil for systemic lupus erythematosus refractory to other immunosuppressive agents

BACKGROUND: Mycophenolate mofetil (MMF) is an immunosuppressive drug widely used in solid organ transplantation, and it may play an increasing role in autoimmune disease. MMF has been introduced as a novel immunosuppressive agent in systemic lupus erythematosus (SLE), often in patients intolerant of or resistant to conventional immunosuppressive regimens. METHODS: We studied 21 patients with SLE, most of whom had previously received courses of cyclophosphamide therapy and had also received courses of azathioprine or methotrexate. Indications for treatment included uncontrolled disease activity and worsening renal involvement. RESULTS: MMF treatment resulted in reduced disease activity, as assessed by the SLEDAI (SLE disease activity index) (P=0.0001) and decreased proteinuria (P=0.027) while allowing a significant reduction in oral corticosteroid dose (P=0.0001). Levels of complement factors C3 and C4 and anti-double-stranded DNA antibodies were not significantly affected. CONCLUSION: MMF appears to be a safe and effective alternative immunosuppressant for extra-renal and renal disease in SLE not responding to conventional immunosuppressive treatment.     

Clinical and laboratory variables of childhood systemic lupus erythematosus in western province of Saudi Arabia

To study the demographic, clinical, laboratory features, treatment and outcome of childhood systemic lupus erythematosus (SLE) in western province of Saudi Arabia. Children with SLE who were diagnosed at King Abdulaziz University Hospital, Jeddah, between March 1998 and October 2008 were included. Charts of all patients were reviewed retrospectively for clinical and laboratory features, treatment and outcome. There were 28 girls and 2 boys, with a mean age of 10.5 years (range 5–18). The female:male ratio was 14:1. Constitutional symptoms represent significant symptoms. Hematological manifestations were the most frequent finding (86.7%) at the time of diagnosis followed by arthritis and nephritis (73.3%). The malar rash represents the most common skin manifestation (46.7%). Discoid lupus was very rare. Neurological symptoms were seen in 30%, while cardiac and pulmonary involvement was uncommon. All patients had positive ANA and 90% of them had high anti-ds DNA. All patients were treated with steroids and hydroxychloroquine and 26 patients received immunosuppressive therapy. Three patients died due to severe infection; massive brain infraction and severe lung disease (one in each). Twenty-seven patients are alive in stable condition. Clinical manifestations and laboratory abnormalities were similar to previously reported series. This report confirms that SLE has comparable findings among children from different ethnicities.     

维生素D受体起始密码子基因多态性与系统性红斑狼疮相关性研究

目的 检测维生素D受体(VDR)基因多态性在系统性红斑狼疮(SLE)患者中的分布,探讨其与SLE发病的相关性.方法 应用聚合酶链反应-限制性片段长度多态性(PCR-RFLP)分析技术检测VDR起始密码子(FokI)多态性位点和基因型在271例SLE患者和130名健康对照组中的分布情况.采用χ2检验和方差分析进行统计学处理.结果 SLE患者及健康对照组VDR FokI多态性基因型和等位基因分布均不处于Hardy-Weinberg平衡(SLE组χ2=7.883,P=0.019;健康对照组:χ2=7.288,P=0.026).VDR FokI多态性等位基因F和f的分布频率在健康对照组分别为48.8%和51.2%,在SLE组分别为60.9%(χ2=10.39,P=0.001)和39.1%(χ2=10.39,P=0.001);F等位基因个体发生SLE的比值比(OR)为1.630(95%CI=1.210～1.1%,χ2=10.39,P=0.001).基因型FF、Ff和ff分布频率在健康对照组中分别为25.4%、46.9%和27.7%,在SLE组中分别为42.8%(χ2=11.417,P=0.001)、36.2(χ2=4.251,P=0.039)和21.0%(χ2=2.187,P=0.139);FF和Ff基因型个体发生SLE的OR分别为2.200(95%CI=1.385～3.493,χ2=11.417,P=0.001)和0.641(95%C1=0.419～0.979,χ2=4.251,P=0.039).进一步分析发现,不同VDR FokI多态性基因型SLE患者之间疾病活动性积分(SLEDAI)差异无统计学意义(P=0.382).但与FF和ff基因型SLE患者对照,Ff基因型SLE患者中浆膜炎的发生率更高(P=0.001),而且具有更高阳性率的抗双链DNA(dsDNA)抗体(P=0.001)、抗Sm抗体(P=0.047)和抗组蛋白抗体(P=0.001),但皮疹发生率较低(P=0.005).结论 VDR FokI多态性位点F等位基因和F/F及F/f基因型与SLE发病易感性有关,而且F/f杂合子患者更容易发生浆膜炎和产生抗dsDNA抗体、抗Sm抗体和抗组蛋白抗体.