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。     

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抗核抗体与临床表现的相关性研究

目的 探讨系统性硬皮病(SSc)患者血清自身抗体与临床之间的关系.方法 对1981-2009年确诊为系统性硬皮病的283例患者的临床资料进行分析.结果 283例患者中,女253例(89.4%),平均发病年龄(35.9±12.6)岁,就诊时平均病程(4.3±4.5)年.弥漫性皮肤型SSc共125例(44.2%).ANA阳性96.8%,抗Scl-70抗体阳性54.4%,ACA阳性6.4%,抗RNP阳性23.7%,抗Sm抗体阳性7.1%,抗SSA抗体阳性25.1%,抗SSB抗体阳性7.1%,抗Jo-1抗体5H性1.1%,未检出抗rRNP抗体.抗Scl-70抗体、ACA均阳性者仪1例.在IcSSe中ACA阳性率较dcSSc中高(P<0.05).结论 抗核抗体检测有助于对SSc患者进行诊断、分型、预后及处理.     

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...     

81例结缔组织病的血清抗核抗体分析

结缔组织病患者具有明显的体液免疫功能异常,有自身抗体 (特别是抗核抗体 )的存在。我们分析了自 1995年以来,我院收治的系统性红斑狼疮 (SLE)、皮肌炎和多发性肌炎 (DM/PM)、系统性硬皮病 (PSS)、干燥综合症 (SS)及混合性结缔组织病 (MCTD)81例。本组病例均为活动期患者,就其抗核抗体的检测情况进行了观察总结,现报告如下。 检测方法：抗 ds- DNA抗体的测定采用胶体金标记和斑点渗滤技术。 ENA多肽抗体的测定采用免疫印迹法,试剂盒均由广州万孚生物技术有限公司提供。 结果： 63例 SLE患者中抗核抗体阳性 52例 (82.54％ )…     

系统性硬化病63例内脏累及与自身抗体检测

目的 探讨系统性硬化病（SSc）的临床特点及内脏累及与自身抗体的相关性。方法 分析2012—2015年就诊于复旦大学附属中山医院皮肤科的63例SSc患者的临床资料、实验室检查及辅助检查,并分析内脏损伤、临床表型与自身抗体的相关性。结果 63例SSc患者中弥漫型与局限型SSc各29例,重叠综合征5例;30例有肺间质纤维化;32例有肺功能受损,表现为一氧化碳弥散量减低;12例肺动脉压升高;29例抗DNA拓扑异构酶Ⅰ（Scl?70）抗体阳性,其中19例（65.52%）有肺间质纤维化;34例抗Scl?70抗体阴性,其中11例（32.35％）有肺间质纤维化;19例抗干燥综合征A（SSA）抗体阳性,其中13例（68.42%）有肺间质纤维化;44例抗SSA抗体阴性,其中17例（38.64％）有肺间质纤维化。抗Scl?70、SSA抗体阳性患者中肺间质纤维化发生率均高于阴性患者（χ2值分别为6.901和4.720,P < 0.05）。63例中22例有手指尖溃疡,其中16例（72.73%）有肺间质纤维化;没有指尖溃疡41例中14例（34.15%）发生了肺间质纤维化,两组肺间质纤维化发生率比较,差异有统计学意义（χ2 = 8.544,P < 0.01）。50例（79.40%）患者以雷诺现象为首发表现,雷诺现象发生后平均48.3个月被确诊为SSc。结论 SSc患者肺间质纤维化发生率高,常伴肺功能受损和肺动脉高压,首发表现多为雷诺现象。指尖溃疡、抗Scl?70、SSA抗体阳性患者肺间质纤维化发生率高。     

结缔组织疾病

960177 149例系统性红斑狼疮患者核抗原多肽抗体谱的检测/李月文…//云南医药。-1995,-16(4).-291 应用免疫印迹技术(IBT)检测149例系统性红斑狼疮(SLE)患者和70例健康人的细胞核内可抽提核抗原(ENA)多肽抗体,结果ENA多肽抗体阳性97例(65.12％),其中以Sm为主,阳性率为33.56％,抗核糖体抗体阳性率为12.1％,70例健康人均为阴性。在Sm阳性的病例中,6例伴有u_1RNP抗体阳性,3例伴有SSA抗体阳性,1例伴有SSB阳性。阳性患者血清在38、16.5、15 KD多肽呈现阳性,抗SSA的检测有助     

系统性硬化病患者的皮肤表现及其临床意义

目的 探讨系统性硬化病（SSc）患者的皮损特点及其与临床分型、自身抗体、内脏器官损害之间的关系。方法 对2012—2014年复旦大学附属中山医院皮肤科及上海市中西医结合医院硬皮病专科确诊的120例SSc患者进行分析。结果 120例中,皮损发生率依次为雷诺现象118例（98.3%）、皮肤硬化116 例（96.7%,指背硬化101例,占84.2%）;皮肤肿胀90例（75.0%,手指肿胀84例,占70%）;皮肤异色症77例（64.2%）、口唇变薄75例（62.5%）、毛细血管扩张74例（61.7%）、口周放射性条纹63例（52.5%）、面具脸57例（47.5%）、甲小皮增生49例（40.8%）、甲皱出血点35例（29.2%）、指尖凹陷25例（20.8%）、指腹萎缩24例（20.0%）、指末节缩短24例（20.0%）、指尖溃疡15例（12.5%）。抗Scl?70抗体阳性42例（35.0%）,抗着丝点抗体阳性31例（25.8%）。手指肿胀、指尖溃疡、指腹萎缩在抗Scl?70抗体阳性组发生率高于抗Scl?70抗体阴性组（P＜0.05）。指背硬化、皮肤异色症、指尖溃疡、指腹萎缩在抗Scl?70阳性组发生率高于抗着丝点抗体阳性组（P＜0.05）。主要内脏器官损害发生率依次为肺间质病变50%（44/88）、心脏受累47.8%（55/115）、肺动脉高压35.7%（41/115）、食管受累28.3%（34/120）、肾脏受累9.2%（11/120）。弥漫皮肤型系统性硬化病（dcSSc）患者心脏受累和皮肤异色症发生率明显高于局限皮肤型系统性硬化病（lcSSc）患者（P＜0.01）。手指肿胀、指背硬化、皮肤异色症、毛细血管扩张、口唇变薄、口周放射性条纹在SSc 患者早期发生率较高,手指肿胀、指背硬化与肺动脉高压发生率相关性较高;毛细血管扩张、指尖凹陷、指尖溃疡与肺间质病变发生率相关性较高;指腹萎缩与心脏累及发生率相关性较高,差异均具有统计学意义。结论 雷诺现象、手指肿胀、指背硬化、皮肤异色症、毛细血管扩张、口唇变薄、口周放射性条纹有助于SSc早期诊断,肺动脉高压在疾病早期即会出现,指尖凹陷、指尖溃疡预示有肺间质病变,指腹萎缩预示有心脏受累。     

ENA多肽抗体谱试剂检测自身抗体

ENA多肽抗体谱所包括的七种自身抗体(Sm,RNP,SSA,SSB,Rib,Scl-70和Jo-1抗体)存在于不同的自身免疫疾病中。有的抗体仅见于某一疾病,有的抗体虽可见于多种疾病,但在不同疾病中的阳性率有明显差异。识别可提取核抗原(ENA)是近年来抗核抗体(ANA)研究的一项重大进展。我们采用ENA多肽抗体谱试剂盒检测了不同自身免疫病患者血清中七种自身抗体,结果报告如下。     

抗Scl-70和抗Jo-1抗体在系统性硬皮病和皮肌炎中的临床意义

为了提高对系统性硬皮病及皮肌炎的临床确诊率，通过ＥＮＡ抗原应用免疫印迹技术检测了１９例系统性硬皮病（ＳＳ）及２８例皮肌炎或多发性肌炎（ＤＭ／ＰＭ）患者抗Ｓｃｌ ７０及抗Ｊｏ １抗体，并与系统性红斑狼疮（ＳＬＥ）、混合性结缔组织病（ＭＣＴＤ）共６２例及５０例健康人作了对照研究。结果表明：７０ ＫＤ（抗Ｓｃｌ ７０）多肽抗体是系统性硬皮病的特异性标记抗体，阳性率３１．６％；５５ＫＤ（抗Ｊｏ １）多肽抗体是皮肌炎的特异性标记抗体，阳性率４６．４％。ＳＬＥ、ＭＣＴＤ及健康人全部阴性，从而有助于对系统性硬皮病及皮肌炎的鉴别诊断     

系统性硬皮病治疗前后血清ANA及ENA抗体检测的意义

目的：探讨ＡＮＡ、ＥＮＡ抗体与系统性硬皮病（ＳＳＤ）转归的关系。方法：免疫印迹法检测７种ＥＮＡ抗体，间接免疫荧光法检测ｄｓＤＮＡ、ＡＮＡ抗体。结果：ＳＳＤ中７种ＥＮＡ抗体阳性率为６４．７％，其中以抗Ｓｃｌ ７０抗体阳性率最高（４１．２％），治疗后抗Ｓｃｌ ７０抗体及抗Ｒｏ部分转阴，ＡＮＡ治疗前阳性率７３．５％，治疗后阳性率及滴度明显下降。结论：ＡＮＡ抗体可作为判断ＳＳＤ病情是否好转的参考指标，抗Ｓｃｌ ７０，抗Ｒｏ部分转阴是否与ＳＳＤ的好转有关，值得进一步观察。     

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.     

Long‐term outcome of patients with polymyositis/ dermatomyositis and anti‐PM‐Scl antibody

Background To date, no series has analysed long‐term outcome in patients with polymyositis/dermatomyositis (PM/DM) with anti‐PM‐Scl antibody. Objectives The aims of the present study were: (i) to assess clinical features and long‐term outcome, including organ complications, functional course and mortality rate, in patients with isolated PM/DM with anti‐PM‐Scl antibody; and (ii) to evaluate prevalence, characteristics and long‐term outcome of interstitial lung disease (ILD) in patients with isolated PM/DM with anti‐PM‐Scl antibody. Methods The medical records of 20 consecutive patients with isolated PM/DM with anti‐PM‐Scl antibody were reviewed. Results Two patients (10%) achieved remission of PM/DM, whereas 14 (70%) improved and four (20%) had a worsened clinical status. Short‐term recurrences (during tapering of therapy) occurred in nine patients and long‐term recurrences (after discontinuation of therapy) in three patients. Moreover, patients with PM/DM with anti‐PM‐Scl antibody exhibited severe complications, as follows: oesophageal involvement (n = 4) requiring enteral feeding in three cases, ventilatory insufficiency (n = 3) requiring mechanical ventilation in two cases; three other patients had cancer. Interestingly, patients with PM/DM with anti‐PM‐Scl antibody often presented symptoms that are usually found in antisynthetase syndrome, i.e. hyperkeratotic rhagadiform hand symptoms (n = 2; 10%), Raynaud’s phenomenon (n = 8; 40%), arthralgia/arthritis (n = 7; 35%) and ILD (n = 12; 60%). In our cohort, the associated ILD often required combined therapy of steroids and immunosuppressive agents. Conclusions Our series suggests that the presence of anti‐PM‐Scl antibody is not a good prognostic factor in patients with PM/DM, as there appears to be an association with lung and oesophageal involvement; in addition, anti‐PM‐Scl antibody may coexist with malignancy in patients with PM/DM. Furthermore, anti‐PM‐Scl antibody‐positive patients with PM/DM often exhibit ‘mechanic’s hands’, Raynaud’s phenomenon and joint involvement. Our latter findings raise the possibility that the immunogenetic background influences the autoantibody status of these patients; HLA‐DR3 has, in fact, been found in association with antisynthetase syndrome antibodies and with anti‐PM‐Scl antibodies.     

Nuclear antibodies as serologic markers in progressive systemic scleroderma

In all, 36 patients with progressive systemic sclerosis (29 women, 7 men) were studied clinically and immunologically; 15 patients had acrosclerosis (type I) and 21, sclerosis extending beyond the wrist (type II). The sera of all patients were evaluated for ANA (HEp-2-cells), Scl-70, centromere and other ENA antibodies. The centromere antigen was characterized by immunoblotting. All patients had high-titer ANA antibodies (100%); 36% of patients had the Scl-70 antibody (a marker antibody for PSS); and in 22% of our patients a centromere antibody was detected. In all cases the anti-centromere sera reacted with a 19.5-kd polypeptide and in 2 cases they reacted with 23- and 25.5-kd proteins in addition. In patients with centromere antibodies there was increased organ involvement (heart, lung, kidney) compared with patients who had anti-Scl-70 or other nuclear antibodies.     

Autoantibody profiles in systemic sclerosis: Predictive value for clinical evaluation and prognosis

Systemic sclerosis (SSc) is thought to be an autoimmune disease, as autoantibodies against a variety of extractable nuclear antigens can be detected in patient sera. Subgrouping patients based on the type of autoantibodies present can be useful in diagnosis and management. Anti-centromere antibodies (ACA) and anti-topoisomerase I antibodies (anti-topo I) are the classic autoantibodies associated with SSc. ACA are associated with limited cutaneous involvement and isolated pulmonary hypertension, whereas anti-topo I are associated with diffuse skin involvement and pulmonary fibrosis. ACA are predictors for a favorable prognosis, while anti-topo I are correlated with a poor prognosis and SSc-related mortality. Additionally, anti-RNA polymerase antibodies (anti-RNAP) are associated with diffuse cutaneous disease and renal involvement. Anti-nucleolar antibodies define multiple subgroups of patients with SSc. Of these, anti-Th/To antibodies (anti-Th/To) and anti-PM-Scl antibodies (anti-PM-Scl) are associated with limited cutaneous SSc (lSSc), whereas anti-U3RNP antibodies (anti-U3RNP) are associated with diffuse cutaneous SSc (dSSc). In addition, anti-Th/To and anti-U3RNP can be predictors for a less favorable prognosis with a higher frequency of organ involvement, such as pulmonary fibrosis, pulmonary hypertension and renal crisis. Other autoantibodies are less frequently reported: anti-Ku antibodies, anti-U1RNP antibodies, anti-human upstream-binding factor, and anti-U11/U12 antibodies. These antibodies are generally less specific to SSc, but also define clinically distinct patient subsets. Thus, characterization of autoantibodies in SSc together with knowledge of disease characteristics intrinsic to distinct patient populations is helpful for assessing the clinical presentation and prognosis of this disease, and for monitoring patients with SSc.     

Demographic and clinical features of systemic sclerosis patients with anti‐RNA polymerase III antibodies

Anti‐RNA polymerase III antibody (RNAP) is primarily detected in diffuse cutaneous type systemic sclerosis (dcSSc) patients and strongly associated with renal crisis. Additionally, there has been increasing evidence that cancer in SSc patients is associated with RNAP. The aim of this study was to examine the demographic and clinical features of SSc patients with RNAP. Among 246 SSc patients, 5.7% were positive for RNAP, 20.7% were positive for anti‐topoisomerase I antibody (Topo I) alone and 39.4% were positive for anticentromere antibody (ACA) alone. The modified Rodnan total skin score (mRTSS) in SSc patients with RNAP (19.1 ± 2.6) was significantly higher than those in SSc patients with Topo I (11.5 ± 1.1) and patients with ACA (4.4 ± 0.4). Furthermore, among SSc patients with RNAP, the levels of RNAP were positively correlated with mRTSS. Renal crisis is also significantly more prevalent in SSc patients with RNAP than patients without RNAP. Male sex, dcSSc subtype, digital vasculopathy, including digital ulcers and acro‐osteolysis, interstitial lung disease and rheumatoid arthritis complications were prevalent in SSc patients with RNAP and patients with Topo‐I. Primary biliary cirrhosis and Sjögren's syndrome were more in SSc patients with RNAP and patients with ACA compared with patients with Topo 1. No significant difference in the frequency of complications, including Raynaud's phenomenon, pulmonary artery hypertension and malignancy was observed between the three groups. Thus, measurement of RNAP in SSc patients is useful for the diagnosis and risk stratification of severe manifestation, such as renal crisis and severe skin sclerosis.     

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.     

Arteriographic evaluation of vascular changes of the extremities in patients with systemic sclerosis

BACKGROUND: Although digital ulcerations frequently occur in patients with systemic sclerosis (SSc), there have been few reports on the macrovascular involvement. OBJECTIVES: To evaluate the macrovascular involvement in patients with SSc exhibiting digital ulceration or gangrene. METHODS: Transfemoral catheter arteriography of the upper and/or lower extremities was performed in eight patients (one man and seven women, age range 42-71 years) with SSc exhibiting digital ulceration or gangrene. The background of the patients, such as autoantibody profiles and vascular risk factors including smoking habits, was also investigated. RESULTS: Macrovascular involvement was detected in seven of eight patients. In three of seven patients who underwent arteriography of the upper extremity, occlusion was limited to the digital arteries. Obliteration of the ulnar artery and superficial palmar arch was detected in three of seven patients, and the radial artery in one patient. Only one of five patients who underwent arteriography of the lower extremity showed the occlusion limited to digital arteries of the foot. Occlusion of the posterior tibial artery, dorsalis pedis artery and arcuate artery was detected, each in one patient. Two patients showed occlusion of the plantar arch. Overall, the occlusion of arteries proximal to the digits was demonstrated in four of eight patients. Three of the four patients were positive for antitopoisomerase-1 antibody and had diffuse cutaneous SSc (dcSSc) with multiple skin ulcers or gangrene. CONCLUSIONS: Macrovascular involvement as detected with arteriography is not rare in SSc patients with digital ulceration or gangrene. Moreover, the vascular occlusion proximal to the digits seemed to be frequent in antitopoisomerase-1 antibody-positive dcSSc patients with multiple skin ulcers or gangrene.