系统性红斑狼疮的外周血淋巴细胞亚群变化和抗淋巴细胞抗体

<正> 系统性红斑狼疮(SLE)是一种侵及机体多系统的自身免疫性疾病,其免疫学异常表现为外周血淋巴细胞数目减少和T细胞功能缺失;自发产生多种抗体,其中包括与淋巴细胞起反应的抗淋巴细胞抗体(ALA)。长期以来学者们一直在寻找其外周血T淋巴细胞亚群变化与淋巴细胞抗体之间的关系,现综述如下。     

流式细胞仪对系统性红斑狼疮患者CD8+T淋巴细胞亚群的检测

目的探讨CD 8T淋巴细胞对系统性红斑狼疮的作用机制.方法用流氏细胞仪直接免疫荧光法检测SLE患者外周血CD3 、CD4 、CD8 、CD28 T淋巴细胞的百分率.结果活动期SLE患者CD3 细胞、CD8 细胞百分率明显高于对照组和缓解期患者,CD4 /CD8 比值亦明显降低(P＜0.05),CD4 细胞百分率比较无统计意义.在CD8 细胞中,CD8 CD28细胞的百分率明显高于对照组和稳定期患者(P＜0.05).稳定期患者,CD3 细胞、CD4 细胞、CD8 细胞、CD4 /CD8 比值与对照组比较均无显著性差异(P＞0.05).在CD8 细胞中,CD8 CD28-细胞所占百分率与对照组比较无统计意义(P＞0.05).结论 SLE活动期患者虽然CD8 细胞增多,但大多数是CD8 CD28-细胞,失去细胞毒性作用.相反,与活动期患者相比,稳定期患者体内生成CD8 CD28 淋巴细胞.CD8 CD28-T细胞在SLE的发病机制中起着一定的作用,且患者体内的CD8 T抑制淋巴细胞的变化是可逆的.     

系统性红斑狼疮患者淋巴细胞CD126 的表达

我们分别采用流式细胞仪结合微量全血双标记免疫荧光法和半定量逆转录聚合酶链反应 (RT-PCR)技术检测了 33例 SLE患者 CD3＋与 CD19＋淋巴细胞表面 CD126表达水平和外周血单一核细胞 (PBMC)内 CD126 mRNA表达水平,现报道如下。 一、材料与方法 (一 )研究对象：符合 1982年美国风湿病协会修订的 SLE诊断标准的患者共 33例,男 6例,女 27例,年龄 17～ 53岁,平均年龄 (32.2± 10.1)岁。参照文献 [1]按受检时的临床表现及实验室检查结果对 SLE疾病活动性（ SLEDAI）进行评分,受检时总分 >9分者判为活动期, 18例,男 3例,女…     

系统性红斑狼疮患者淋巴细胞凋亡的研究

目的　研究系统性红斑狼疮患者 (SL E)淋巴细胞凋亡情况以及自身抗体产生的关系。方法　通过分离出淋巴细胞 ,在 12孔板内培养 0、2 4、48小时 ,用吖啶橙染色 ,于荧光显微镜下观察计数。结果　 2 2例 SL E患者的淋巴细胞凋亡率较 16例正常对照组在培养 2 4、48小时时有显著增高 (P均 <0 .0 1)。SL E患者伴有 2种以上自身抗体阳性者 ,培养 2 4、48小时具有更高的淋巴细胞凋亡率 (P<0 .0 5和 P<0 .0 1)。结论　 SL E患者淋巴细胞在体外凋亡加速 ,可能与体内自身抗体产生增多有关 ,其淋巴细胞凋亡异常在其发病机制中起一定作用。     

系统性红斑狼疮患者外周血T淋巴细胞的激活状态

目的探讨系统性红斑狼疮(SLE)患者外周血中T淋巴细胞激活状态及其在发生发展过程中所起的作用。方法收集本院门诊和住院治疗的60例SLE患者设为SLE组,另有40例于同期来本院查体的健康体检者设为正常对照组。采用双色流式细胞术测定SLE患者组和正常对照组外周血CD38/CD3分子水平;放免法和间接免疫荧光法检测其血清抗ds DNA抗体;间接免疫荧光法检测其抗核抗体;免疫散射比浊法检测其补体C3和C4水平;速率酶法检测血清肌酐水平。再根据疾病活动指数评分结果将SLE组患者分为活动期组和稳定期组,根据相关标准还分为肾炎组和非肾炎组。结果 SLE患者外周血CD38/CD3分子水平(724±139分子/细胞),显著高于健康对照组(641±110分子/细胞);活动期SLE患者CD38/CD3分子水平(905±138分子/细胞)显著高于非活动期患者(671±111分子/细胞);狼疮肾炎患者CD38/CD3分子水平(911±146分子/细胞)显著高于非肾炎患者(681±127分子/细胞),差别均具有统计学意义(P均<0.05)。狼疮患者CD38/CD3分子水平与肌酐水平(r=0.75,P<0.01)、抗ds DNA抗体(r=0.78,P<0.01)及SLEDAI评分(r=0.67,P<0.01)显著相关,而与C3和C4水平(r=-0.19,-0.24;P>0.05)无相关性。结论 CD38/CD3分子水平与反应SLE活动度的指标密切相关,T淋巴细胞激活状态在SLE特别是狼疮肾炎的发生、发展中可能起重要作用。本研究仅对女性患者进行检测分析,CD38/CD3分子水平与SLE男性患者疾病发生发展的相关性还有待进一步研究。     

系统性红斑狼疮患者T淋巴细胞亚群Fas及bcl-2抗原的表达

目的　通过对 SL E患者末梢血 CD4 、CD8 、CD45 RO T淋巴细胞亚群表面 F as及 bcl-2抗原表达的测定 ,寻找与 SL E发病有关的因素。方法　用流式细胞仪双色解析法。结果　与健康对照组比较 ,SL E患者的 CD4 、CD8 、CD45 RO T淋巴细胞亚群表面 Fas抗原表达明显增强 (P<0 .0 1) ,而 bcl-2抗原表达明显降低 (P<0 .0 1)。结论　末梢血 CD4 、CD8 、CD45 RO T淋巴细胞亚群表面 F as抗原表达增强 ,bcl-2抗原表达降低即过度细胞凋亡现象可能与 SL E发病有关。     

系统性红斑狼疮患者外周血淋巴细胞性激素受体检测

很多资料表明性激素水平与系统性红斑狼疮(SLE)发病有关,而性激素必须与特异性受体结合才能发挥出生物学效应,其生物学效应放大既取决于性激素浓度,又取决于受体的数量和质量。因此我们测定SLE患者及正常人血清性激素水平,同时应用免疫组化方法检测了外周血淋巴细胞性激素受体。     

自身基因,淋巴细胞凋亡与系统性红斑狼疮

自身基因缺陷造成洒巴细胞凋亡异常，与自身免疫在性疾病的发生，发展和治疗关系极为密切。该文对调控淋巴细胞凋亡相关的自身基因及其在系统性红斑狼疮中的作用综述如下。     

ELEVATED LEVELS OF CHEMOTAXIS INHIBITORY ACTIVITY IN SERA OF PATIENTS WITH SYSTEMIC LUPUS ERYTHEMATOSUS

An inhibitory activity against neutrophil chemotactic factors was found in normal human serum. Elevated levels of this chemotaxis inhibitory activity were demonstrated in sera of patients with systemic lupus erythematosus (SLE). The physico-chemical characteristics of this chemotaxis inhibitory substance(s) in SLE serum were almost identical to those of the inhibitory substance(s) in normal human serum; both the inhibitory substances were heat labile, soluble in 45% saturated ammonium sulphate, worked directly on chemotactic factor and affected various chemotactic factors. A study using Sephadex G- 100 chromatography showed that the molecular sizes of both inhibitory substances were almost identical. No correlation was found between the levels of the chemotaxis inhibitory activity in SLE sera and other clinical parameters including erythrocyte sedimentation rate, and serum concentration of either the third component of complement (C3) or the fourth component of complement (C4). Specimens from patients with the following diseases failed to demonstrate elevated levels of chemotaxis inhibitory activities; rheumatoid arthritis, urticaria, psoriasis vulgaris, atopic dermatitis and discoid lupus erythematosus (DLE).     

SIGNIFICANCE OF IN VIVO-BOUND IMMUNOGLOBULINS AND COMPLEMENT IN THE SKIN OF SYSTEMIC LUPUS ERYTHEMATOSUS PATIENTS

A direct immunofluorescent test was used to demonstrate the in vivo deposits of immunoglobulin (Ig) and complement in the skin of 30 patients with systemic lupus erythematosus (SLE). These deposits were demonstrated in all skin lesions and in 70% of apparently normal skin of patients with lupus nephritis. On the contrary, they were seldom found in seemingly normal skin of patients without nephritis, though they could be seen in skin lesions. There was significant difference between these two groups and as well as an intimate relation of the deposits to clinical severity. The fixed Ig and/or complement could be found in a band arrangement in the basement membrane zone (BMZ) area, nuclear areas of epidermal and/or dermal cells or both BMZ area and nuclear areas.     

DIAGNOSTIC EVALUATION OF THE LUPUS BAND TEST IN DISCOID AND SYSTEMIC LUPUS ERYTHEMATOSUS

Background. The usefulness of the lupus band test (lbt ) in the diagnosis of cutaneous lupus erythematosus remains controversial. The study was done to determine the sensitivity, specificity, and predictive value of the lbt in discoid lupus erythematosus (dle ) and systemic lupus erythematosus (sle ). Methods. During the study period, 32 patients with SLE and 28 patients with DLE were included. The final classification of cases was based on the American Rheumatism Association Criteria (ara ) for sle , histopathology, and consensus of the clinical staff. Thirty controls, 15 each for DLE and sle , were chosen from a group of patients, who had lesions clinically simulating dle and sle , but could be excluded by histopathology, laboratory tests, and follow-up. Histopathologic examinations and direct immunofluorescence (DIF) tests were done on lesional skin. Results. In dle , the sensitivity of the LE band test was 58% and the specificity 87%. The positive predictive value was 95% and negative predictive value was 32%. In sle the sensitivity was 93% and specificity 87%. The positive predictive value was 64% and negative predictive value 98%. Conclusions. The high negative predictive value of lbt in sle suggests that it is valuable in excluding diseases clinically similar to sle .     

血小板和血管内皮细胞在系统性红斑狼疮出血倾向中的意义

本文从血小板和血管内皮细胞方面对系统性红斑狼疮(SLE)出血倾向进行探讨,结果发现活动期SLE除存在外周血小板破坏过多,骨髓巨核细胞代偿增生外,同时有血浆TXB_2含量减低、血小板聚集功能异常;还存在内皮细胞产生的活性物质(6-Keto-PGF_1α,vWf:Ag和Fn)显著升高(P均<0.01)。并探讨了其发生可能的机理和临床意义。     

DIRECT IMMUNOFLUORESCENT STUDY OF SYSTEMIC LUPUS ERYTHEMATOSUS IN SINGAPORE*

The direct immunofluorescent antibody technique was employed in the study of 33 patients with systemic lupus erythematosus (SLE), 51 patients with miscellaneous dermatoses and 12 normal controls. Positive “band” test was found in 14 patients, 11 of whom had SLE. The “band” was observed in 41 per cent of SLE involved skin biopsies and 32 per cent of the SLE uninvolved skin biopsies. The major immune complex components in the eleven “band” positive SLE cases were, in order of frequency, IgM, IgG, complement (C₃), IgA and fibrinogen. A majority (63.4 per cent) demonstrated the “homogenous” band pattern which was commonly associated with deposition of IgM and IgG. “Thready” band pattern was present in a third of the positive biopsies and was formed mainly by IgG and complement (C₃). “Stippled” band pattern was seen in four biopsies only. “Band” positive patients had a higher incidence of systemic involvements and autoantibodies, and a poorer prognosis than the negative group. Comparisons are made of the above findings with those reported in other series. The results and other findings are then discussed.     

CUTANEOUS MANIFESTATIONS OF SYSTEMIC LUPUS ERYTHEMATOSUS IN A TERTIARY REFERRAL CENTER

Background:

Systemic lupus erythematosus (SLE) is an autoimmune disease with multiorgan involvement. The skin is the second most commonly affected organ. SLE with skin lesions can produce considerable morbidity resulting from painful skin lesions, alopecia, disfigurement, etc. Skin lesions in patients with lupus may be specific (LE specific) or may be non specific (LE non specific). Acute cutaneous LE (Lupus specific) has a strong association with systemic disease and non-specific skin lesions always indicate disease activity for which patients present to rheumatologists and internists. Therefore, a thorough understanding of the cutaneous manifestations of SLE is essential for most efficient management.

Aims:

The aims of this study were to evaluate the patterns and prevalence of skin lesions in patients with SLE and to assess the relationship between skin lesions and other systemic involvement.

Materials and Methods:

At the Department of Rheumatology and Clinical Immunology, IPGME&R in Kolkata, 150 patients with SLE fulfilling the clinical and laboratory criteria of the American Rheumatology Association (updated 1982) were examined and followed-up for cutaneous manifestations between January 2002 and January 2007.

Results:

Skin lesions were important clinical features. About 45 patients (30%) presented with skin lesions although all patients had skin lesions during the follow-up period. Skin changes noted were as follows: Lupus specific lesions: malar rash in 120 patients (80%), photosensitive dermatitis in 75 patients (50%), generalized maculopapular rash in 40 patients (26.67%), discoid rash in 30 patients (20%), subacute cutaneous lupus erythematosus (SCLE) in 5 patients (3.34%), lupus profundus in 5 patients (3.34%). The lupus non-specific lesions were non-scarring alopecia in 130 patients (86.67%), oral ulcers in 85 patients (56.67%), vasculitic lesions in 50 patients (33.34%), bullous lesions in 15 patients (10%), Raynaud''s phenomenon in 10 patients (6.67%), pyoderma gangrenosum in 2 patients (1.34%), erythema multiforme in 10 patients (6.67%), and nail fold infarcts in 2 patients (1.34%); however, mucosal discoid lupus, lichenoid discoid lupus, livedo reticularis, sclerodactyly, etc. were not detected. Patients having lupus-specific skin lesions e.g., malar rash were associated with systemic involvement, whereas those having lupus non-specific skin lesions were associated with disease flare.

Conclusions:

Skin lesions in patients with SLE are important disease manifestations and proper understanding is essential for diagnosis and efficient management.