【作者】 李昕；

【导师】 巩路；

【作者基本信息】 天津医科大学 ， 内科风湿病学， 2004， 硕士

【摘要】 系统性红斑狼疮(SLE)是一种由免疫复合物介导和沉积引起的自身免疫性炎症性疾病。它常常累及全身多数器官。本病好发于育龄女性，男女发病率之比为1：8～10。遗传、环境、免疫及性激素是其发病因素。感染和大量毒素的作用是系统性红斑狼疮的触发机制。我们已知，SLE易感基因是SLE发病的基础，自身免疫耐受的破坏、多克隆自身反应性淋巴细胞增生以及自身抗体的产生是SLE发病的中心环节。近年来，随着对本病早期诊断以及有效治疗的开展，本病的预后得到显著改善。 目的： 本课题通过对280例系统性红斑狼疮住院患者临床病例进行回顾性分析，旨在探讨SLE的流行病学和临床特征，评估常规实验室检查指标在SLE的诊断、判断病情活动程度以及评定治疗反应中的意义。同时调查SLE患者合并重要脏器损害和感染并发症的情况以及死亡原因。从而可以帮助我们进一步认识SLE的发病机制和临床特征以及有效指导临床治疗改善患者预后。 方法： 回顾性调查自1999年4月～2004年3月在我科住院的280例SLE患者，对年龄、性别、病程持续时间、首次发病年龄、首发症状、常规实验室检查指标、合并感染、重要脏器损害的情况以及死亡原因等项目列表登记，利用统计学方法得出相关结论。 结果： 1．一般情况：280例患者中，男21例，女259例，男女比例为1：12。年龄最小13岁，最大者74岁，平均年龄37.82±16.27岁。平均发病年龄33.46±11.95岁。病程最短14天，最长20年，平均病程3.4±1.6年。平均住院时间为23.3±4.8天。所有统计病例中活动期患者192例，稳定期患者88例；初诊未经系统治疗的患者144例，已经过系统治疗的患者136例。 天津医科大学硕士研究生学位论文 2.临床特征:发热是最常见的首发症状，共有191例，占68.21%，多数呈中等度热。合并重要脏器损害包括:肾脏受累占据首要位置，占31.43%，心血管系统占25%;血液系统占2421%;肺损害占23.44%;中枢神经系统占9.38%;肝脏损害占6.25%。 3.实验室检查指标: (l)血清抗核抗体(ANA)总阳性率为98.21%，其中高滴度(ANA妻l:犯0)阳性率为60%，低滴度(ANA<1:200)阳性率为3821%，有5例患者呈现ANA阴性。 (2)活动期组患者血清抗双链DNA(dsDNA)抗体和抗Sm抗体阳性率高于稳定期组，经统计学检验有显著差别(P<0.01)。 (3)活动期组免疫球蛋白IgG升高、补体C3降低、血白细胞(wBC)减低以及血小板(PLT)减低的患者比率高于稳定期组，经统计学检验均有显著差别(PLT尸<0.05，其余指标P<0.01)。C反应蛋白(CRP)升高的患者比率在两组间比较则无显著差别(尸>0.05)。 (4)治疗前组dsDNA阳性率，补体C3下降、WBC减低以及PLT减低的患者比率高于治疗后组，经统计学检验均有显著差别(尸<0.01)。 4.SLE合并感染的情况:在所有住院患者中，合并感染的患者为166例，占59.38%。 (1)呼吸道感染占首位，为60乃3%，其后依次为泌尿生殖系、消化道、皮肤及软组织、败血症、中枢神经系统感染。 (2)在166例次感染病例中，细菌感染109例次，占65.79%，其次为真菌和病毒感染，各占13.86%和13.25%。 ③经过系统治疗患者组的感染率高于初诊患者组，经统计学检验有显著性差别(P<0.01)。 (4)老年组(鑫60岁)感染率高于青年组(基20岁)，经统计学检验有显著性差别(尸<0.01)。 (5)低白蛋白血症组的感染率高于血浆白蛋白正常组，经统计学检验有显 天津医科大学硕士研究生学位论文著差别(尸<0.05)。 (6)近期(发生感染前2周内)应用抗生素组的感染率高于未用抗生素组，经统计学检验有显著差别(尸<0.01)。 (7)应用大剂量肾上腺糖皮质激素组的感染率高于中小剂量组，经统计学检验有显著差别(尸<0.05)。 (8)激素与环磷酞胺联合应用组的感染率高于未联用组，经统计学检验有显著差别(尸<0.01)。 (9)白细胞减低组与白细胞正常组的感染率经统计学检验无显著差别(尸>0 .05)。 5 .SLE患者的死亡原因主要为疾病活动、感染和重要脏器损害。死亡患者中伴发感染的比例为66.67%，因感染直接导致死亡的病例为5例，占27.78%。重要脏器损害造成的死亡以肾脏和中枢神经系统多见，也有因心血管损害和血栓栓塞性疾病造成的死亡情况发生。结论: 1.不明原因的发热是SLE患者的最主要的首发症状，它应被作为SLE的重要的临床特征之一，应将SLE作为不明原因发热的重要鉴别诊断之一。 2. dsDNA、IgG、补体C3、wBC、PLT以及抗sm抗体在狼疮活动期中的意义非常重要。可以作为判断SLE病情活动与否及对治疗反应进行评定的重要指标。 3.老年SLE患者较年轻患者更易伴发感染。低白蛋白血症、近期(发生感染前2周内)应用抗生素、大剂量应用肾上腺糖皮质激素以及激素与环磷酞胺联合应用造成了SLE患者合并感染率的明显升高。中小剂量激素和血白细胞减低不会提高SLE患者的感染率。 4.感染因素在SLE的各个阶段都是非常重要的导致死亡的因素。同时要重视对SLE患者伴发动脉粥样硬化、急性心脑血管事件以及血栓栓塞疾病的预?更多还原

【Abstract】 Systemic lupus erythematosus (SLE) is a chronic inflammatory autoimmune disease characterised by the mediation and the deposition of immune complexes in various tissues. It always invades many organs in body. It has a 1:8~10 F:M ratio and high rate of occurrence in women of childbearing. Various hormonal,genetic,immunologic and environmental factors have been associated with an increased risk for its development. Infectious agents and variousvaccines may act as a trigger mechanism for the induction of autoimmunity and the development of SLE.As we know,genes in susceptibility to SLE are the basementto generate SLE, the disorder of immunologic tolerance, polyclonal self-activationlymphocyte and the produce of autoantibody play a central role in development of SLE. The prognosis for lupus has been greatly improved in recent years owing to earlier diagnosis and an effective remedy.Objective:Our retrospective clinical analysis of in-patients with SLE is going to explore the clinical and epidemiologic characteristics of SLE.We also assess the utility of measurement of laboratory parameters in the diagnosis,evaluating the dasease activity and the reaction to therapy.In our study ,we are in order to investigate thesituation of organ damage , infectious complication and the cause of the death inSLE.So we can establish deep conception on the truth of the diease and guide the clinical therapy in order to improve the prognosis.Methods:Retrospective investigate 280 cases of SLE were admitted to our department5since 1999.4 to 2004.3.Register the data of the age,sex,clinical manifestation of onset,duration of the disease, routine laboratory data, the situation of systemdamage , infection complication and the cause of the death in SLE.Drow acorrelation conclusion by statistical analysis.Results:1 .Demographic details: The study cohort comprised two hundreds and fifty-nine female and twenty-one male patients,the F:M ratio was l:12.The avarge age was 37.82+16.27 years. The average age of the oncet of the disease was 33.46+11.95,a mean follow-up of 3.4+1.6 years.The mean duration in hospital was 23.3 + 4.8 days.The number of the patients with active-stage was one hunred and ninty-two,the number of the patients with inactive-stage was eighty-eight.2.Clinical features.’The oncet manifestation was fever,it was always moderate fever,it’s frequence was 68.21%. The cumulative frequencies of organic involvement were: renal 31.43% was in the first place,vascular 25%, haematological 24.21%, lunged 23.44%,neurological 9.38%, and hepatic 6.25%.3. Laboratory data:(1) The positive frequence of ANA was 98.21%, the percentage of high-titre(i?1:320) ANA was 60%,the low-titre( < 1:200) ANA was 38.21%.(2) Camparied with the patients with inactive-stage ,the positive percentage of serum anti-dsDNA and anti-Sin was higher in the patients with active-stage,it had significant difference (P < 0.01) .(3) Camparied with the patients with inactive-stage,high level of serum IgG (P < 0.01) and low level of WBC (/>< 0.01) ,PLT (P< 0.05) and complmentC3(P < 0.01 )in the patients with active-stage had significant difference.But serum level of CRP had no significant difference (P>0.05) .(4) Comparied with the patients after therapy, the abnormal percentage ofserum anti-dsDNA,WBC,PLT and complment Cj in the patients before therapy6had significant difference (P < 0.01) .4.The situation of SLE complicated infection: The total number of the patients with complicated infection was 166, its percentage was 59.38% 0(1) The position of infection:Respitatory infection was in the first place among the whole infection cases,its percentage was 60.53%,the others also involved genitourinary,gastrointestinal,skin and mucosal, septicemia and central nervous system infection.(2) The aetiology of infectionrthe percentage of bacterial infection was 65.97%,the percentage of fungi and virus infection were 13.86% and 13.25%.(3) Comparied with the patients first visiting to hospital,the percentage of infection in p更多还原