系统性硬皮病的循环抗体及发病机理

一、系统性硬皮病的循环抗体系统性硬皮病患者可发现循环自身抗休。用间接免疫荧光,以人类喉部肿瘤细胞     

银屑病患者抗肿瘤坏死因子α治疗前后血清抗核抗体、抗dsDNA抗体及抗ENA抗体的变化

目的：观察银屑病患者接受抗肿瘤坏死因子α制剂治疗后抗核抗体（ANA）、抗dsDNA抗体和抗可提取性核抗原（ENA）抗体的变化。方法回顾分析32例银屑病患者,其中13例使用英夫利西单抗治疗,19例使用依那西普治疗。英夫利西单抗组第0、2、6周各用药1次,此后每隔8周用药,于每次用药前检测患者ANA、抗dsDNA抗体及ENA的情况和临床症状的变化。依那西普组每周用药2次,每3~6个月检测患者ANA、抗dsDNA抗体及ENA的情况和临床症状的变化。采用银屑病皮损面积和严重度指数（PASI）75、疾病活动评分（DAS）28评估临床疗效,间接免疫荧光法检测血清ANA水平,免疫印迹法和ELISA法检测抗dsDNA抗体水平,免疫印迹法检测抗ENA抗体水平。结果32例银屑病患者临床症状有不同程度缓解。32例抗TNF?α治疗的患者中有7例（21.9%）出现自身抗体,其中英夫利西单抗组中4例治疗（8.3±5.1）个月后出现自身抗体,3例ANA阳性,3例ENA阳性;依那西普组中3例治疗（9.0±3.0）个月后出现自身抗体,3例ANA阳性,1例ENA阳性。结论部分银屑病患者接受抗肿瘤坏死因子α制剂治疗后可出现自身抗体。     

ELISA技术检测寻常型天疱疮桥粒芯蛋白3抗体水平研究

目的:评价酶联免疫吸附试验(ELISA)检测抗桥粒芯蛋白(desmoglein,Dsg)3抗体在寻常型天疱疮(PV)诊断中的意义.方法:对来自不同中心的106例PV患者和106例对照人群血清标本编盲后,进行ELISA检测抗Dsg3自身抗体和间接免疫荧光(IIF)法检测血清天疱疮自身抗体.结果:ELISA法检测Dsg3抗体敏感度为77.4%,特异性为94.3%;IIF法检测抗体敏感度为79.2%,特异性为94.3%.两组间的差异无统计学意义.结论:ELISA方法检测Dsg3抗体对于寻常型天疱疮的诊断是一种较好的辅助方法.     

抗角蛋白自身抗体的研究动态

<正> 应用免疫吸附、混合血球凝集,间接血球凝集试验及间接免疫荧光技术(ⅡF),早已发现人血清中存在着抗角质层的自身抗体,这种自身抗体不仅出现于银屑病为主的多种皮肤疾患,而且几乎见     

抗桥粒芯蛋白抗体对天疱疮的临床诊断价值

目的研究天疱疮患者血清中抗桥粒芯蛋白(desmoglein,Dsg)1和抗Dsg3抗体水平在天疱疮的临床诊断价值。方法采用酶联免疫吸附试验(ELISA)测定62例天疱疮患者血清中抗Dsg1和抗Dsg3抗体水平;间接免疫荧光法(IIF)测定天疱疮抗体Ig G水平。结果 62例天疱疮患者中抗Dsg1抗体或抗Dsg3抗体任一阳性共50例,阳性率80.65%,与间接免疫荧光法(IIF)相比两种方法差异无统计学意义(P0.05)。因此抗Dsg抗体具有和IIF一样的临床诊断价值。34例寻常型天疱疮(PV)中抗Dsg3抗体阳性或抗Dsg1和抗Dsg3抗体均阳的28例,阳性率82.35%;24例落叶型天疱疮(PF)中抗Dsg1抗体阳性的为20例,阳性率83.33%。可见抗Dsg3抗体是PV的鉴别诊断指标,抗Dsg1抗体是PF的鉴别诊断指标。此外,通过对治疗前后抗Dsg1抗和Dsg3抗体的滴度水平的检测发现,抗Dsg1和抗Dsg3抗体还可作为天疱疮治疗监测的指标。结论采用ELISA方法测定天疱疮患者外周血抗Dsg1抗体和抗Dsg3抗体,方法简便,对患者创伤小,有一定的诊断和鉴别诊断价值。另外,抗Dsg1抗体和抗Dsg3抗体还可作为监测天疱疮治疗效果的指标。     

血浆疗法治疗大疱性类天疱疮的临床疗效及自身抗体水平变化

目的:通过研究大疱性类天疱疮(bullous pemphigoid, BP)患者的临床资料,提高对大疱性类天疱疮的认识,并初步评价血浆疗法治疗大疱性类天疱疮的临床疗效,并探索其治疗机制。方法:2009年12月—2015年12月我院皮肤科收治的大疱性类天疱疮患者66例,28例患者接受血浆疗法联合糖皮质激素治疗,作为血浆疗法组(PT组),38例患者单纯应用糖皮质激素治疗,作为单纯糖皮质激素治疗组(HT组),比较两组的临床疗效,每组各采集16名患者的血清,以20名健康者血清作为对照,采用酶联免疫吸附(ELISA)法检测治疗前后血清中抗BP180抗体水平的变化以及抗BP180抗体亚型IgG1、IgG4水平的变化。结果:PT组有效率为92.86%,HT组为73.68%,PT组的治疗效果更为显著。两组患者治疗后抗BP180抗体滴度明显下降(P0.01),PT组较HT组下降明显(P0.01),PT组患者治疗后抗体亚型IgG1、IgG4测得的吸光度A值明显下降(P0.05)。结论:血浆疗法治疗大疱性类天疱疮有效,其机制可能与降低抗BP180抗体及抗体亚型IgG1、IgG4的水平有关。     

抗中性粒细胞胞浆抗体在系统性红斑狼疮中的临床意义

目的 为了探讨抗中性粒细胞胞浆抗体(ANCA)在系统性红斑狼疮(SLE)中的临床意义.方法 通过间接免疫荧光(IIF)、酶联免疫吸附试验和免疫印迹方法检测了84份SLE患者血清ANCA的阳性率和6种已知的靶抗原:蛋白酶3、髓过氧化物酶、杀菌-通透性增高蛋白、弹力蛋白酶、组蛋白酶G和乳铁蛋白.结果 间接免疫荧光法检测ANCA在SLE中的阳性率是226%,均为核周型ANCA;分别有44.1%和14.3%的SLE患者血清含有抗组蛋白酶G和乳铁蛋白自身抗体,且活动期的阳性率明显高于稳定期.结论 组蛋白酶G为ANCA的主要靶抗原,且抗组蛋白酶G抗体与SLE的病情活动有关.     

间接免疫荧光检测抗肌内膜抗体确诊疱疹样皮炎

采用间接免疫荧光法检测1例两次皮损周围皮肤直接免疫荧光检查阴性、临床疑似疱疹样皮炎的24岁女性患者的血清,结果显示患者血清抗肌内膜自身抗体1:20阳性,结合临床诊断为疱疹样皮炎;随后第三次对皮损周围皮肤行直接免疫荧光检查发现典型的真皮乳头部位IgA颗粒状沉积.     

血清抗桥粒芯蛋白1和3抗体在天疱疮临床诊断中的意义

目的探讨血清抗桥粒芯蛋白(desmoglein,Dsg)1抗体和抗Dsg3抗体检测在天疱疮诊断中的临床意义。方法用酶联免疫吸附试验(ELISA)检测拟诊为天疱疮的113例患者血清中的抗Dsg1抗体和抗Dsg3抗体,同时行皮损组织病理以及直接免疫荧光和间接免疫荧光检查。结果 77例(68.14%)患者经组织病理、直接免疫荧光和间接免疫荧光检查确诊为天疱疮,其中Dsg1抗体阳性71例,阳性率62.83%,敏感度为92.21%,特异度100%;Dsg3抗体阳性45例,阳性率39.82%,敏感度58.44%,特异度100%;二者均阳性39例(34.51%)。其余36例(31.86%)患者经组织病理、直接免疫荧光和间接免疫荧光检查确诊为其他大疱性皮肤病,Dsg1抗体和Dsg3抗体均为阴性。结论 ELISA方法测定外周血抗Dsg1抗体和抗Dsg3抗体具有较高的敏感度和特异度,方法简便,对患者创伤小,对天疱疮临床诊断和鉴别诊断具有重要参考价值。     

大疱性类天疱疮患者血清中抗BP180抗体水平检测及意义的分析

目的研究23例大疱性类天疱疮(BP)患者血清中抗BP180抗体的表达水平和治疗前后的变化及抗体指数与病情严重程度的相关关系,以探讨抗BP180抗体水平在BP患者的诊断、监测病情变化及治疗中的应用。方法对23例患者通过病理及直接免疫荧光进行诊断,并对患者的病情进行评估,应用酶联免疫吸附试验(ELISA)法检测患者及20例正常人血清中抗BP180抗体的水平,最后通过统计分析判断各指标间的关系及意义。结果23例患者血清经ELISA检测抗BP180抗体阳性18例;治疗后的抗BP180抗体指数(67.94±54.73)较治疗前(74.22±59.06)下降,治疗前与治疗后的差值为3.66±12.71,差异有统计学意义(P<0.05);治疗前后抗BP180抗体指数均与病情评分有相关性(治疗前r=0.417,P<0.05;治疗后r=0.818,P<0.01)。20例正常对照组阳性1例,阴性19例。ELISA检测BP血清抗BP180抗体的灵敏度为78.26%,特异性为95%。结论BP患者血清中抗BP180抗体水平与疾病的严重程度有一定的相关性,有较高的灵敏度和特异性,可作为BP的常规辅助诊断,并用于监测病情变化及指导治疗。     

Pemphigus

Pemphigus is an infrequent, organ-specific, autoimmune bullous disease, which affects the skin, mucous membranes and appendages. Histopathologically, it is characterized by acantholysis. Pemphigus has classically been divided into two major groups, pemphigus vulgaris and pemphigus foliaceus, with their respective clinical variants pemphigus vegetans and pemphigus erythematosus. In recent years, new variants of pemphigus have been described: paraneoplastic pemphigus, IgA pemphigus and pemphigus herpetiformis. This article reviews the epidemiology, etiopathogenesis, clinical symptoms, diagnosis, treatment and prognosis of pemphigus. Advances in molecular biology techniques have made it possible to more precisely identify the different antigens against which antibodies are directed, and to fine-tune ELISA diagnostic techniques. Treating pemphigus vulgaris and foliaceus with general steroids has modified their prognosis; it is estimated that mortality in recent decades is less than 10 %. Managing the clinical complications that appear during the evolution of the pemphigus has contributed to reducing morbidity and mortality.     

Pemphigus

Pemphigus is an autoimmune intraepithelial blistering disease of the skin and mucous membranes that is characterized in part by the presence of circulating IgG autoantibodies. These autoantibodies, which have been shown to be pathogenic, bind to complexes on the keratinocyte cell surface and have been utilized to establish that these complexes contain components found in cell adhesion junctions. Although the stimulus for autoantibody production is unknown, proposed mechanisms to explain the disease pathophysiology include proteinase activation, complement activation, and direct interference of cell adhesion junction assembly by autoantibodies. Although pemphigus vulgaris was commonly fatal prior to the availability of glucocorticosteroids, the prognosis has improved dramatically since their introduction. Immunosuppressive agents have also been successfully employed to treat patients with more severe disease.     

Pemphigus

Pemphigus refers to a group of potentially life‐threatening autoimmune diseases of the skin and mucous membranes, characterized by the formation of blisters and erosions of the skin. An autoimmune process, directed against keratinocyte desmosomal cadherins, interferes with the adhesive function of these molecules. This results in the separation of keratinocytes and clinical manifestation of blistering. Differences in the particular antigens targeted by the antibodies and in the distribution of these antigens in the different regions of the body and in the separate layers of the epidermis result in different clinical manifestations of the disease. Diagnosis of pemphigus is based on three independent groups of criteria: clinical features (flaccid blisters and erosions on skin and oral mucosa), histologic findings (epidermal acantholysis) and immunological tests (circulating and skin‐fixed antibodies against keratinocyte surface antigens). The principle aim of treatment is to reduce inflammatory response and autoantibody production, thereby achieving disease remission. Systemic corticosteroids are still the most useful drugs in the treatment of pemphigus and continue to be the mainstay of therapy for this disease. Adjuvant drugs, such as immunosuppressants, are commonly used in combination, in order to increase efficacy and have a steroid‐sparing effect, thereby allowing reduced maintenance doses and less side effects of systemic corticosteroids. Other options include intravenous immunoglobulin and plasmapheresis. However, more research is needed to develop treatments with the least possible toxicity.     

Pemphigus

Pemphigus is a group of organ-specific autoimmune mucocutaneous disorders with an established immunologic basis. The presence of intraepithelial blisters and erosions of the skin and variable involvement of the mucous membranes characterize its three major variants, pemphigus vulgaris, pemphigus foliaceus, and paraneoplastic pemphigus. Prior to the use of corticosteroids in the 1950s, the natural history of pemphigus vulgaris was relentless progression, with a 50% mortality at 2 years, and almost 100% at 5 years. Today, with mortality rates less than 5%, the focus has changed towards reducing corticosteroid side effects and maintaining optimal quality of life under treatment. This can be achieved by the appropriate use of steroid-sparing agents. This article addresses the comprehensive management of patients with pemphigus.     

Pemphigus

The term pemphigus refers to a group of autoimmune intraepidermal blistering diseases of the skin and mucous membranes. Several clinical variants of pemphigus are recognized. The major histologic feature of all variants is acantholysis, the disruption of normal cell-to-cell adhesion, which leads to intraepidermal blister formation. Most patients with pemphigus demonstrate IgG autoantibodies directed against an antigen located on the surface of keratinocytes. Although the stimulus for autoantibody production is unknown, several mechanisms have been proposed to explain the pathogenesis of acantholysis. One popular model proposes that pemphigus antibodies induce acantholysis through local stimulation of the plasminogen-plasmin system. Another model proposes that pemphigus antibodies fix complement and thereby alter cell membrane integrity to produce acantholysis. Prior to the availability of corticosteroids, pemphigus vulgaris was commonly fatal. Treatment with glucocorticosteroids has drastically improved the prognosis. Immunosuppressive agents and plasmapheresis have been used successfully in some patients with severe disease.