系统性硬化症患者血清自身抗体与临床和预后的相互关系

作者对397例系统性硬化症(硬皮病)患者作了血清抗 Scl-70及抗着丝粒抗体(ACA)的测定。发现102例(26%)患者 Scl-70抗体阳性,86例(22%)患者 ACA 阳性,无1例患者两种抗体均阳性。ACA 几乎都(96%)见于 CREST 综合征的患者,但大多数(57%)局限性硬皮病患者没有这种抗体。有此抗体的这些患者更常有钙化和毛细血管扩张,很少有肺间质纤维化和限制性的肺疾     

抗Scl-70抗体与系统性硬化病临床特征的相关性研究

目的:探讨抗Scl-70抗体与系统性硬化病(SSc)临床特征的相关性.方法:回顾性分析1990年1月-2018年2月该院142例SSc住院患者资料,包括性别、年龄、临床表现和实验室检查等.采用IBM SPSS Statistics 23.0软件对抗Scl-70抗体阳性和抗Scl-70抗体阴性SSc患者临床特征的差异进行...     

系统性硬皮病抗U1RNP抗体的检测及其临床意义

系统性硬皮病（SSc）患者体内存在着多种自身抗体,如抗Scl-70、抗U1RNP、抗着丝点抗体和抗RNA聚合酶抗体。国内外对抗U1RNP抗体与SSc的相关性研究较少。笔者发现SSc患者抗U1RNP抗体阳性率较高,故对314例SSc患者作了抗可提取核抗原（ENA）抗体检测,并就抗U1RNP抗体阳性患者的临床表现作一分析．探讨该抗体在SSc中的临床意义。     

系统性硬皮病患者血清ENA多肽抗体与临床的关系

分析46例系统性硬皮病(SSc)患者血清ENA(盐水可提取抗原)多肽抗体与临床的关系。结果表明ENA多肽抗体中 ,抗Scl 70抗体与肺间质纤维化紧密相关 ,检测SSc患者抗Scl 70抗体有助于估计其器官累及情况。早期不发生器官累及的皮肤弥漫型系统性硬皮病(dcSSc)可称为慢性dcSSc。     

局限性硬皮病的抗核抗体

作者报告了用间接免疫荧光法测定22例局限性硬皮病患者的抗核抗体(ANA)的结果,发现用Hela细胞作底物阳性率达72.7%(16/22),其中硬斑病为50%(2/4);泛发性硬斑病为100%(6/6);线状硬皮病为66.7%(8/12).而用小鼠肾作底物时阳性率为50%(11/22),其中泛发性硬斑病为100%(6/6),线状硬皮病为33.3%(4/12),硬斑病为25%(1/4).所有病例均未发现抗Sm,nRNP,Scl-70和SS-B抗体.4例泛发性硬斑病抗双链DNA抗体阳性,但结合活性比SLE要低得多.用组蛋白重结合技术检测抗组蛋白抗体,全部病例阴性.     

抗着丝点抗体阳性自身免疫性疾病患者临床及免疫学特征分析

目的:分析抗着丝点抗体(ACA)阳性自身免疫性疾病患者特征。方法:回顾性分析我院皮肤科2020年1月至2021年6月间接免疫荧光法检测ACA自身免疫性疾病的患者,分为ACA阳性组和ACA阴性组,比较两组性别、年龄、临床诊断、抗核抗体谱、免疫学指标、临床表现等。结果:共分析ACA阳性自身免疫性疾病患者53例,ACA阴性患者31例。ACA阳性组平均年龄(59.85±11.79)岁高于ACA阴性组(49.06±16.60)岁(P<0.05);ACA阳性组高滴度(≥1∶1000)、抗CENP-B抗体、抗AMA-M2抗体阳性占比分别为98.11%、94.34%和22.64%,高于ACA阴性组的77.42%、6.45%和6.45%(P_s<0.05);ACA阳性组血清IgG水平(11.22±5.36)g/L低于ACA阴性组(P<0.05);ACA阳性患者雷诺现象(43.40%)、心血管受累(33.96%)发生率高于ACA阴性组(P<0.05)。结论:ACA阳性的自身免疫性疾病患者具有发病年龄晚、ACA检出滴度高,易出现雷诺现象、心血管受累,抗AMA-M2...     

抗线粒体Ⅱ型抗体与抗着丝点B蛋白抗体重叠阳性在CREST综合征中的临床意义

采用间接免疫荧光(IIF)法、酶免疫斑点(ELISPOT)法和免疫印迹(Western blot)法检测107例进行性系统性硬皮病(SSc)患者,血清中的ANA阳性率及核型、AMA阳性率及亚型AMA-M2、AMA-M4和AMA-M9的阳性率、CENP-B和Scl-70的阳性率,分析AMA-M2和CENP-B在CREST综合征中的重叠阳性率,同时记录AMA-M2和CENP-B 重叠阳性患者的临床症状和相关实验室指标,探讨AMA-M2与CENP-B重叠阳性在CREST综合征中的临床意义.     

系统性硬皮病患者抗Scl—70抗体的测定及临床意义

用免疫印迹技术检测并分析49例系统性硬皮病患者血清ENA（盐水可提取核抗原）多肽抗体与临床的关系。结果表明，ENA多肽抗体中抗Scl-70抗体与肺间质纤维化和指（趾）端缺血紧密相关。皮肤弥漫型系统性硬皮病患者的内脏病变与皮肤改变的严重程度并不平行。     

抗着丝粒抗体和抗局部同分异构酶Ⅰ抗体与临床的联系

为进一步研究抗着丝粒抗体(ACA)和抗局部同分异构酶I(topoisomerase I,简称topo I)抗体与临床的联系,作者对355例病人的血清进行了研究。其中89例为近心端硬皮病,54例CREST综合征,154例原发或继发性雷诺氏病。另58例患SLE、干燥综合征或RA,但无雷诺氏现象,任何CREST 表现、皮肤变厚或发紧,这些病人作为结缔组织病(CTD)的对照组。34名健康人为另一对照组。以HEp-2细胞作底物,用间接免疫荧光法测ACA;以免疫扩散法测抗topo I(Scl-70)抗体;以提纯的HeLa 细胞染色体作为底物,用免疫印迹法检测ACA 及抗topo I。结果:①CTD 对照组及健康人对照组ACA 阴件,仅1名健康人用免疫印迹法检测抗topo I 抗体,结果阳性,CTD 对照组未检出此抗体。用两     

日本系统性硬皮病患者临床与预后的关系

系统性硬皮病(SSc)是一影响结缔组织的自身免疫性疾病,其免疫功能异常的标志是血清抗核抗体(ANA)的出现,90％以上的SSc患者ANA阳性。本文通过回顾性研究观察各种ANA与临床特征以及预后的相关性。 275例患者来自1971～1990年日本Keio大学医学院的初诊患者,均符合美国风湿病学会关于SSc的诊断标准。观察2年,在初诊的第一年内采集每个患者的血清标本,检测8种与SSc相关的ANA。     

Clinical significance of antinuclear antibodies in Japanese patients with systemic sclerosis

Clinical significance of specific antinuclear antibodies (ANA) in Japanese patients with systemic sclerosis was studied. The patients with systemic sclerosis were classified into four groups according to ANA: (1) anticentromere antibody-positive; (2) anti-Scl-70 antibody-positive; (3) anti-nRNP antibody-positive, and (4) others. The mean score (the number of positive signs in six selected specific signs) of the patients with anti-Scl-70 antibody was significantly higher than those of the other three groups. More frequent contracture of phalanges in the patients with anti-Scl-70 antibody, less diffuse pigmentation in the patients with anti-nRNP antibody, and less pulmonary fibrosis in the patients with anticentromere antibody were revealed. As shown above, the detection of specific ANA in systemic sclerosis is clinically important.     

Scl 70 antibody—a specific marker of systemic sclerosis

Scl 70 antibodies were tested for in 107 patients with systemic sclerosis: 68 with acrosclerosis and 39 with diffuse scleroderma. Anticentromere antibodies (ACA) and other antinuclear antibodies (ANA) were tested for by indirect immunofluorescence on HEp-2 cells. Positive results for Scl 70 antibodies were obtained in 77% of cases of diffuse scleroderma and 44% of acrosclerosis. ACA and Scl 70 antibodies were found to be mutually exclusive. If acrosclerosis cases positive for anticentromere antibodies are excluded, the percentage of acrosclerosis cases positive for Scl 70 was 63%. ACA were found to be a marker of a benign, abortive subset of acrosclerosis with almost no cutaneous involvement (CREST), whereas Scl 70 did not discriminate between acrosclerosis and diffuse scleroderma. On HEp-2 cells Scl 70 positive sera gave a characteristic, fine speckled, almost homogeneous nuclear staining pattern.     

Autoantibodies other than antineutrophil cytoplasmic antibodies are not positive in patients with psoriasis vulgaris

There is a great deal of evidences that a link may exist between psoriasis and autoimmunity. In this study, thirty-six patients with psoriasis vulgaris and twenty healthy controls were screened for antinuclear antibody (ANA), anti-ds-DNA antibodies, anti-SSA (anti-Ro), anti-SSB (anti-La), anti-Scl-70, anti-Jo-1, rheumatoid factor (RF), C3 and C4 levels, IgG and IgM anti-cardiolipin antibodies (aCL) and anti-neutrophil cytoplasmic antibodies (pANCA and cANCA) in order to determine the role of autoimmunity in the pathogenesis. In addition, the MPO-ANCA test was performed on pANCA-positive patients. In ANA screening, one patient showed 1/80 granular positivity. No positive results were obtained from either the study or control groups for anti-dsDNA, anti-SSA, anti-SSB, anti-Scl-70, or anti-Jo-1 determinations. For RF, 5.8% of the patients had positive values. Both C3 and C4 levels were found to be significantly elevated in psoriasis patients compared to healthy controls (p<0.001). IgG-aCL and IgM-aCL positivity was not significantly different between the two group. pANCA was positive in 33.3% of the patients and a significant difference was observed between study and control groups (p<0.05). MPO-ANCA was negative in all the pANCA-positive patients. In conclusion, only serum C3 and C4 levels and pANCA determinations showed significant differences when compared to healthy controls. Our findings may be evidence of associations between autoimmunity and psoriasis vulgaris. However, more detailed studies in this field need to be done to determine the relationship between them.     

The clinical relevance of serum antinuclear antibodies in Japanese patients with systemic sclerosis

BACKGROUND: Systemic sclerosis (SSc) is a connective tissue disorder with excessive fibrosis of the skin and various internal organs. Although SSc is a heterogeneous disease, it has been reported that the particular antinuclear antibodies (ANA) are often indicative of clinical features, disease course and overall severity. OBJECTIVE: To clarify the association of clinical and prognostic features with serum ANA in Japanese patients with SSc. METHODS: We studied 203 Japanese patients diagnosed with SSc, who visited our hospital during the period 1983-2005. Six SSc-related ANA were identified using indirect immunofluorescence, double immunodiffusion and immunoprecipitation assays. RESULTS: Patients with SSc were classified into six ANA-based subgroups and a group without ANA. As expected, antitopoisomerase I antibody (Ab, n=64), anti-RNA polymerases (RNAP) Ab (n=12) and anti-U3 RNP Ab (n=5) were associated with diffuse cutaneous SSc, whereas anticentromere Ab (ACA, n=75), anti-Th/To Ab (n=7) and anti-U1 RNP Ab (n=10) were frequently detected in patients with limited cutaneous SSc. Clinical features of the ANA-negative group (n=10) were heterogeneous. Consistent with previous findings in Caucasian and/or black African patients, antitopoisomerase I Ab was associated with the involvement of vascular and pulmonary fibrosis, leading to decreased survival rate. However, no patients with anti-RNAP Ab developed renal crisis and the frequency of isolated pulmonary hypertension in patients with ACA, anti-Th/To Ab or anti-U3 RNP Ab was similar to that in other ANA-based subgroups. CONCLUSION: These results indicate that the clinical relevance of SSc-related ANA in Japanese patients differs in some aspects from that in Caucasian and/or black African patients.     

Antinuclear antibodies in the relatives of patients with systemic sclerosis

Antinuclear antibodies were studied by indirect immunofluorescence and double immunodiffusion in 21 patients with systemic sclerosis and 35 of their relatives. When HE_p-2 cells were used as the substrate, the frequency of antinuclear antibodies in the patients' sera was 100% and that in the relatives was 26%. When rat liver sections were used, the values were 86% and 17%, respectively. Anticentromere antibody was detected in the serum from the mother of one patient whose serum had anti-Scl-70 antibody. Antibody to n-RNP was positive in the sera from the brother and daughter of another patient whose serum was positive for anti-n-RNP and anti-Scl-70 antibodies. The high frequency of antinuclear antibodies in the sera from the relatives of systemic sclerosis patients suggests that immunological abnormalities play a part in the pathogenesis of this condition.     

抗心磷脂抗体和免疫异常在青斑样血管炎中的意义

目的探讨抗心磷脂抗体(ACA)和免疫异常在青斑样血管炎(LV)中的作用。方法两组均用药前取静脉血,用间接免疫荧光、免疫印迹、ELISA等方法测定LV患者体内抗核抗体、循环免疫复合物、免疫球蛋白、ACA、抗β2糖蛋白1(β2GP1)抗体的水平。结果LV组4例抗核抗体阳性,3例免疫复合物阳性,2例免疫球蛋白升高,其中2例诊断为系统性红斑狼疮。ACA阳性13(43.33%)例,β2GP1水平增高9(30.00%)例,其中3例合并下肢网状青斑样改变。对照组ACA阳性1例,无抗β2GP1抗体阳性者。两组ACA及抗β2GP1抗体阳性率差异有显著性意义(P均<0.01)。结论LV的发病与免疫复合物、免疫反应无明显相关性,ACA与LV血栓形成有关,与合并严重网状青斑患者的ACA和抗β2GP1抗体尤其相关。说明LV的发病与血凝异常及血栓形成有一定关系。     

Screening and studies of the specificity of antinuclear antibodies in lupus erythematosus and scleroderma sera in the tumor cell monolayer substrate

The comparative study of the human tumour cell line HeLa and rat liver sections for the detection of antinuclear antibodies by the indirect immunofluorescence technique demonstrates the superiority of HeLa monolayer in sensitivity and specificity. Use of monolayers is essential for the diagnosis of antinucleolar and anticentromere ANA specificities and permits differentiation between anti-Sm/RNP, anti-SS-B and anti-Scl-70. ANA profiles are evaluated in 142 sera of 72 patients with different forms of lupus erythematosus and scleroderma and in 216 sera of healthy subjects.     

Systemic sclerosis with sarcoidosis: Case report and review of the published work

Sarcoidosis and systemic sclerosis (SSc) rarely coexist. Here, we report a Japanese female SSc patient who developed systemic sarcoidosis. Her SSc was a limited type negative for anti‐Scl‐70 antibody and positive for anticentromere antibody (ACA). Moreover, we performed a review of the English‐language published work that described cases of concurrent SSc and sarcoidosis. Then, we found that most SSc and sarcoidosis concurrent patients positive for anti‐Scl‐70 antibody were male (77.8%). On the other hand, most patients positive for ACA were female (87.5%). These results suggest some relationships between autoantibody profiles and sex in SSc and sarcoidosis concurrence.     

Anti-Scl-70 antibodies in systemic scleroderma

We looked for anti-Scl-70 and anti-centromere antibodies in 109 patients (26 men and 83 women). Mean age was 43 +/- 15 years. Forty patients had systemic sclerosis according to ARA criteria. The extension of cutaneous involvement was defined by using Barnett and Coventry criteria: 12 patients were type I (sclerodactyly), 20 type II (acrosclerosis) and 8 type III (diffuse scleroderma). Among the 12 patients with type I, there were 8 cases of CREST syndrome defined as follows: presence of sclerodactyly, Raynaud's phenomenon and 2 of the 3 following criteria: oesophageal dysmotility, calcinosis, telangiectasia. Other organ involvement was recorded. Control patients had idiopathic Raynaud's phenomenon (n = 22), other connective tissue diseases (n = 20), and miscellaneous diseases (n = 28). Ninety-nine patients were prospectively included in this study. Patients' sera were stored at -20 degrees C. Ten previously stored sera obtained from patients with systemic sclerosis were also analyzed. Immunological tests were performed simultaneously and with no information on the diagnosis. When antinuclear antibodies were detected by indirect immunofluorescence, double immunodiffusion and immunoblotting were performed. Anti-Scl-70 antibodies were detected in systemic sclerosis only: 1 of 12 type I, 11 of 20 type II and 4 of 8 type III. One serum negative by immunodiffusion was positive using immunoblotting. We found that the specificity of anti-Scl-70 antibodies for systemic sclerosis was 100 p. 100 and their sensitivity 40 p. 100. There was a correlation between the presence of anti-Scl-70 antibodies and the presence of antinuclear antibodies (p less than 0.05) and the extent of cutaneous involvement (p less than 0.05).(ABSTRACT TRUNCATED AT 250 WORDS)     

The significance of anticardiolipin antibody and immunologic abnormality in livedoid vasculitis

Objective To evaluate the significance of anticardiolipin and immunologic abnormality in the livedoid vasculitis (LV). Methods 30 patients with biopsy‐proven LV and 30 normal controls involved in the study. Indirect immunofluorescence, immunoblot, and ELISA were used for detecting antinuclear antibody (ANA), circulating immune complex, immune globulin, anticardiophospholipin antibody (ACA), and anti‐β₂GP1. Results ANA was positive in four patients with LV, and among them, two patients were diagnosed as Systemic Lupus Erythematosus (SLE) later. Addition to the two SLE patients, the level of ENA and immunoglobulin were normal in the rest of patients. Anticardiolipin antibodies were present in 13 (43.33%), and β₂GP1 was present in nine (30%) of 30 patients. There were significant differences between LV and controls. Conclusions ACA is one of important pathogenesis of LV. Numerous heterogeneous coagulation abnormalities and thrombogenesis may involve the LV.