结缔组织疾病

单核细胞趋化蛋白-1与系统性红斑狼疮；系统性红斑狼疮患者Toll样受体9基因多态性的初步研究；抗ABCA1自身抗体在SLE患者动脉粥样硬化发病机制中的意义；PDCs在狼疮肾炎患者外周血和肾组织中的表达；不同给药途径时抗核抗体独特型多肽对狼疮样肾炎小鼠作用的探讨；儿童系统性红斑狼疮的进展。     

系统性红斑狼疮患者血清几种趋化因子的检测

目的 探讨血清趋化因子单核细胞趋化及活化因子/单核细胞趋化蛋白-1(MCAF/MCP-1)、正常T细胞表达和分泌的活化调节蛋白(RANTES)水平与系统性红斑狼疮疾病活动程度的关系及意义。方法 采用双抗体夹心ELISA法检测血清MCAF、RANTES水平。结果 ①SLE患者血清MCAF水平明显高于正常人;②活动期患者血清MCAF水平明显高于非活动期;③肾损组与非肾损组相比,血清MCAF、RANTES水平差异均无显着性。结论 趋化因子MCAF可能参与系统性红斑狼疮的发病机制,且血清MCAF水平可作为反映疾病活动性的一个指标。     

结缔组织疾病

Toll样受体9在SLE患者产生抗dsDNA抗体中的作用；系统性红斑狼疮患者血浆和血细胞DNA中p16基因启动子区甲基化现象和意义；SLE患者T淋巴细胞穿孔素mRNA及其蛋白的表达；系统性红斑狼疮患者外周血白细胞介素15的研究；rST细胞对系统性红斑狼疮患者外周血单核细胞活化和凋亡的影响；     

趋化性细胞因子及受体与系统性红斑狼疮

随着免疫学和分子生物学的发展 ,越来越多的趋化性细胞因子及其受体被发现。近年来 ,自从趋化性细胞因子受体被发现作为HIV感染的协同受体后 ,趋化性细胞因子研究领域引起广泛的关注。现综述白介素 - 8、单核趋化蛋白 - 1以及其他几种趋化性细胞因子及其受体与系统性红斑狼疮的关系 ,并提出其在系统性红斑狼疮治疗中的设想     

趋化性细胞因子及受体与系统性红斑狼疮

随着免疫学和分子生物学的发展，越来越多的趋化性细胞因子及其受体被发现。近年来，自从趋化性细胞因子受体被发现作为HIV感染的协同受体后，趋化性细胞因子研究领域引起广泛的关注。现综述白介素－8、单核趋化蛋白－1以及其他几种趋化性细胞因子及其受体与系统性红斑狼疮的关系，并提出其在系统性红斑狼治疗中的设想。     

趋化因子与系统性红斑狼疮的关系

趋化因子（chemokines）是一类能够调控细胞定向迁移的细胞因子,广泛存在于白细胞、单核细胞、巨噬细胞、T细胞和B细胞,并且通过与相应的趋化因子受体结合从而介导对白细胞的趋化作用。一种趋化因子能与多个趋化因子受体相结合,而一个趋化因子可能有多个高亲和性受体,它们共同构成复杂的网络系统,在系统性红斑狼疮的发生发展中起到重要作用。本文主要对不同趋化因子及受体与系统性红斑狼疮关系进行综述。     

神经肽CGRP对银屑病单核细胞趋化功能的调节

为探讨神经肽对银屑病免疫细胞的调节，及其在银屑病神经免疫发病机制中的作用，本研究利用体外细胞培养技术分离培养单核细胞，分别加入外源性神经肽降钙素基因相关肽（calcitonin gene-related peptide,CGRP)及其受体拮抗剂CGRP8－37。用ELISA检测培养单核细胞上清液中趋化因子的含量；利用微型趋化小室，观察CGRP对单核细胞趋化活性的调节。结果CGRP诱导银屑病活化的单核细胞分泌趋化因子巨噬细胞炎性蛋白－1α(macrophage inflammatory protein-1α，MIP-1α）和单核细胞趋化性蛋白－1α（monocyte chemotactic protein-1α,MCP-1α)增加，受体拮抗剂CGRP8－37则抑制这种诱导作用，同时CGRP促进单核细胞对淋巴细胞和中性粒细胞的趋化活性，用CGRP8－37后则趋化活性减弱。提示银屑病皮损内神经肽CGRP可以通过受体诱导单核巨噬细胞分泌MIP－1α和MCP－1α趋化因子，使淋巴细胞和中性料细胞在局部皮损区定向迁移与聚集，促进局部炎性细胞的浸润。     

银屑病患者皮损局部朗格汉斯细胞数量异常机制的研究

目的：探讨银屑病患者皮损局部朗格汉斯细胞(LCs)数量异常的原因。方法：培养银屑病患者皮损处角质形成细胞，通过微孔小室实验检测其上清液对单核细胞的趋化功能；通过酶联免疫吸附法(ELISA)检测上清液中单核细胞趋化蛋白—1(MCP-1)的表达。结果：银屑病患者皮损处角质形成细胞分泌上清液对单核细胞的趋化能力明显强于正常对照组；其分泌的MCP-1水平也高于正常人。结论：银屑病角质形成细胞表达的趋化因子趋化了更多数量的单核细胞至皮损局部，因此，银屑病患者皮损局部LCs的数量理应是增多的。但由于银屑病角质形成细胞表达的其它一些因子也促使了单核细胞衍生的LCs的活化，活化的LCs会迁移至淋巴结或活化后凋亡，又导致其数量减少。因此，银屑病患者皮损局部朗格汉斯细胞数量是一动态变化过程。     

C—myc原癌基因与系统性红斑狼疮

Ｃ－ｍｙｃ原癌基因是一种能编码关键性调控蛋白，与细胞生长、分化增殖有关的正常细胞基因。国外研究发现，在系统性红斑狼疮患者体内Ｃ－ｍｙｃ基因表达有改变，推测该基因可能通过参与免疫细胞异常活化和增殖的调控，构成系统性红斑狼疮发病的重要分子生物学基础。     

寻常性银屑病患者血清单核细胞趋化蛋白-1、巨噬细胞炎性蛋白-1α和巨噬细胞衍生的趋化因子水平测定

近年研究显示,免疫-炎症机制在银屑病发病中起着重要作用,其中β趋化因子家族(又称CC趋化因子)的研究日益受到重视,单核细胞趋化蛋白-1(MCP-1)、巨噬细胞炎性蛋白-1α(MIP-1α)和巨噬细胞衍生的趋化因子(MDC)能够趋化或激活T细胞,同时也是T细胞分泌和表达的调节因子,了解其在银屑病患者血清中的水平有助于进一步认识疾病发生的机制.我们应用酶联免疫吸附(ELISA)法对寻常性银屑病患者血清MCP-1、MIP-1α以及MDC的水平进行测定,现将结果报道如下.     

SLE患者血清、尿液和肾脏骨桥蛋白的检测及临床意义

目的 探讨骨桥蛋白(OPN)在SLE患者血清、尿液中的含量及与SLE脏器损害、活动性指标的关系.方法 收集100例SLE患者临床资料,ELISA方法检测OPN在100例SLE患者和30例正常人对照外周血清中的浓度,同时用ELISA检测OPN在57例SLE患者和15例正常人对照尿液中的浓度.免疫组化检测3例SLE患者肾脏组织中OPN蛋白的表达.结果 OPN在SLE患者血清和尿液中浓度显著升高,分别为(64.03±72.87)μg/L和(454.87±231.63)μg/L,与正常对照组[(29.88±11.28)μg/L,(122.67±39.47)μg/L]相比,差异有统计学意义(P<0.05), SLE活动组血清和尿液中OPN浓度显著升高,分别为(80.92±87.49)μg/L和(584.36±207.15)μg/L,与SLE非活动组[(36.43±23.48)μg/L,(281.08±131.92)μg/L]相比,差异有统计学意义(P<0.05).血清和尿液中OPN浓度均和SLEDAI积分呈正相关(r=0.462,0.901,P值均<0.01).尿液OPN浓度和尿免疫球蛋白G、尿微量白蛋白、尿α1微球蛋白、尿B2微球蛋白呈正相关(r=0.458,0.359,0.342,0.409,P值均<0.05).OPN在狼疮性肾炎患者肾小管上皮表达.结论 OPN与SLE及肾损有密切关系.     

Annular Lupus Vulgaris Mimicking Tinea Cruris

Cutaneous tuberculosis is an infrequent form of extrapulmonary tuberculosis. It is often clinically and histopathologically confused with various cutaneous disorders. A 36-year-old man attended our clinic with slowly progressive, asymptomatic, annular skin lesions on both the thighs and buttocks for 10 years. He consulted with many physicians and was improperly treated with an oral antifungal agent for several months under the diagnosis of tinea cruris, but no resolution of his condition was observed. A diagnosis of lupus vulgaris was made based on the histopathologic examination and the polymerase chain reaction assay. Anti-tuberculosis therapy was administered and the lesions started to regress.     

Prognosis of patients with systemic lupus erythematosus and discoid lesions

BACKGROUND

It has been observed that patients with systemic lupus erythematosus and discoid lesions have a milder systemic disease.

OBJECTIVE

To compare the clinical, demographic and autoantibody profile of systemic lupus erythematosus patients with and without discoid lesions.

METHODS

We carried out a retrospective study involving 288 systemic lupus erythematosus patients who met at least four classification criteria of the American College of Rheumatology for systemic lupus erythematosus, comparing the clinical, serological and demographic factors between patients with and without discoid manifestations.

RESULTS

Of the 288 patients, 13.8% had discoid lesions. Univariate analysis found no differences in the prevalence of malar rash, photosensitivity, arthritis, serositis, leukopenia, lymphopenia and hemolytic anemia or anemia of the central nervous system (p = ns). Renal lesions were more common in those without discoid lesions (p =0.016), and hemolysis (p<0.0001) was more common in those with discoid lesions. Regarding the profile of autoantibodies, only the anti-RNP antibody was more common in those with discoid events (p =0.04). In a logistic regression study, only the renal lesions and anti-RNP maintained their associations with discoid manifestations.

CONCLUSION

Patients with lesions of systemic lupus erythematosus and discoid lesions have lower prevalence of renal involvement and a greater presence of anti RNP.     

转化生长因子β基因在BXSB狼疮小鼠肾脏中的表达

目的研究转化生长因子（ＴＧＦ β）与狼疮性肾炎进展的关系。方法采用同位素标记ｃＤＮＡ探针Ｎｏｒｔｈｅｒｎ印迹杂交方法,检测了自发狼疮性肾炎的ＢＸＳＢ小鼠肾脏组织中ＴＧＦ βｍＲＮＡ水平。结果发现与狼疮性肾炎发病较轻的６月龄雌性ＢＸＳＢ小鼠相比较,狼疮性肾炎发病较重的６月龄雄性ＢＸＳＢ小鼠肾脏表达ＴＧＦ βｍＲＮＡ的量显著增加（Ｐ＜０．０１）,是对照雌鼠表达量的６倍。结论提示ＴＧＦ β可能在狼疮性肾炎肾小球细胞外基质的病理性积聚和肾小球硬化的形成过程中起重要作用。     

Fas抗原及Fas配体在红斑狼疮患者皮损中的表达

目的 研究Ｆａｓ抗原及Ｆａｓ配体（Ｆａｓ－Ｌ）在红斑狼疮皮损中的表达情况。方法 应用免疫组化技术检测２５例系统性红斑狼疮（ＳＬＥ）及１４例盘状红斑狼疮（ＤＬＥ）不同病程的皮损中Ｆａｓ抗原及Ｆａｓ－Ｌ的表达。结果 ＳＬＥ及ＤＬＥ早期皮损朊细胞Ｆａｓ抗原表达强度显著高于正常皮肤（Ｐ〈０．０１）,与病程呈负相关（Ｐ〈０．０５）,且真皮中单一核细胞亦有Ｆａｓ抗原表达;红斑狼疮患者皮损及正常皮肤的角朊细胞均     

系统性红斑狼疮脱发的研究进展

脱发是SLE常见的临床表现之一。SLE脱发可表现为多种类型,如狼疮发、非瘢痕性斑状脱发、弥漫性休止期脱发、盘状红斑狼疮型脱发等,不同类型的脱发在临床表现和组织病理学方面有其各自的特点。SLE脱发与疾病活动性有一定的相关性。目前SLE脱发的发病机制尚未明确,自身免疫性炎症和血管炎造成的局部微环境的改变、毛发营养不良和毛囊周期失调均有可能参与其中。     

Comedogenic lupus: a rare variant of chronic cutaneous lupus erythematosus – case series

BackgroundComedogenic lupus is an uncommon variant of cutaneous lupus, clinically characterized by the presence of comedones, papules and erythematous-infiltrated plaques, cysts and scars in photo-exposed areas, mimicking acne vulgaris and acneiform eruptions.ObjectivesTo report clinicopathological characteristics of patients with comedogenic lupus in a tertiary dermatology service over a 15-year period and review cases described in the literature.MethodsRetrospective study of patients with clinical and histopathological diagnoses of comedogenic lupus between the years 2006 and 2021. The literature search was carried out in the PubMed and VHL Regional Portal databases, using the terms: “comedogenic lupus” and “acneiform lupus” in Portuguese and English.ResultsFive patients were diagnosed during the described period, all female, with a mean age of 56.6 years. Smoking was observed in three cases, as well as pruritus. The most affected site was the face, especially the pre-auricular, malar and chin regions. Follicular plugs, epidermal thinning and liquefaction degeneration of the basal layer were predominant histopathological findings. Hydroxychloroquine was used as the first-line treatment; however, other medications were used, such as dapsone, methotrexate, tretinoin cream, and topical corticosteroids. The literature search identified 17 cases, with a mean age of 38.9 years, 82% of which were women. Only 23% had a diagnosis of systemic lupus erythematosus. Hydroxychloroquine was the most recommended systemic medication.Study limitationsRetrospective, single-center study. The literature search was carried out in two databases.ConclusionsDermatologists should be aware of acneiform conditions with poor response to the usual treatment. Early diagnosis and treatment reduce the risk of unaesthetic scars.     

Pathogenesis of lupus dermatoses in autoimmune mice. XIX. Attempts to induce subepidermal immunoglobulin deposition in MRL/Mp-+/+ mice

The deposition of immunoglobulin (Ig) at the dermo-epidermal junction (DEJ) of the skin, frequently observed in autoimmune mouse strains, is similar to that seen in patients with systemic lupus erythematosus (SLE). MRL/Mp-lpr/lpr (MRL/lpr) mice have an autosomal recessive mutant gene, lpr, which produces massive T-cell proliferation and accelerates the onset of autoimmune diseases. MRL/Mp-+/+ (MRL/n) mice lack the lpr gene, and do not develop autoimmune disease during the first year after birth under pathogen-free conditions. To verify the mechanisms of subepidermal Ig deposition in the skin of LE, we designed an experiment in which we could induce Ig deposition in the control MRL/n mice. Intraperitoneal injection of lymphoproliferative cells of aged MRL/lpr mice induced splenomegaly and splenic granulomatous angitis in the control MRL/n mice. Lipopolysaccharide (LPS), a polyclonal B-cell activator, induced slight splenomegaly and relatively high levels of serum Ig. Dermatopathological investigation revealed mild lymphocyte infiltration without positive Ig deposition at the DEJ of MRL/n mice treated with proliferative T cells. Injection of both proliferative T cells and LPS induced 50% positivity of subepidermal Ig deposition, and high levels of serum immunoglobulins and anti-double stranded DNA (anti-dsDNA) antibodies. These changes were not observed in MRL/n mice injected with thymocytes of newborn MRL/lpr mice. Skin lesions and lupus nephritis were not demonstrated in any of the mice tested. This study suggest that both the mild inflammatory reaction and the presence of anti-dsDNA antibodies are required for the induction Ig deposition at the DEJ in the skin of LE patients.     

结节性皮肤狼疮黏蛋白病1例

患者男,30岁。面部及耳廓鳞屑性粘着红斑,前胸、双上肢大小不等暗红色斑块、结节,部分中央有暗红色萎缩。ESR38mm/h,ANA(+)1∶320,Sm(-),dsDNA(+),抗SSB抗体(+),抗Rib.p抗体(2+)。皮损组织病理可见真皮血管周围淋巴细胞浸润及黏蛋白沉积。结合该患者病情,对结节性皮肤狼疮黏蛋白病临床特点及发病机制进行文献复习。     

新生儿红斑狼疮5例分析

目的探讨新生儿红斑狼疮(NLE)的诊断和治疗方法。方法对5例NLE患儿的临床表现及实验室检查进行分析,并复习相关文献。结果患儿皮损类似SCLE,局限或全身分布,以颜面、躯干及四肢末端为重,患儿及其母亲ANA、抗Ro/SSA及/或抗La/SSBIgG抗体均阳性,心电图检查未见心脏传导阻滞,未经治疗数周后自然消退。结论根据皮损表现和实验室检查可诊断新生儿红斑狼疮,一般不需治疗。