血清Jo—1抗体与皮肌炎／多发性肌炎的临床意义探讨

对35例皮肌炎/多发性肌炎（DM/PM）患作回顾性分析,比较抗Jo-1抗体阳性的DM/PM患临床特点的差异,结果发现抗Jo-1抗体阳性间质性肺病,关节炎,恶性肿瘤和Raynaud现象的伴发率均明显高于抗Jo-1抗体阴性,且起病较急,病情进展快,需要大量的皮质类固醇激素才能控制病情,提示抗Jo-1抗体阳性的DM/PM患病情重,预后差,临床治疗应积极,及时,皮质类固醇激素的维持时间应足够长。     

皮肌炎／多发性肌炎患者血清Jo—1抗体检测的临床意义

应用免疫印迹技术测定５２例皮肌炎和多发性肌炎病人血清可提取性核抗原（ＥＮＡ）多肽抗体，其中Ｊｏ－１抗体的阳性率分别为１３．３％和３１．８％。１１例Ｊｏ－１抗体阳性病人中７２．７％起较急对类固醇治疗反应差，减药或停药易于复；４５．５％伴发间质性肺病，５４．５％伴有Ｒａｙｎａｎｄ现象，６３．６％合并关节炎，３６．４％合并恶性肿瘤。４１例Ｊｏ－１抗体阴性病人上述合并症阳性率仅分别为９．８％、１７．１％、     

多发性肌炎和皮肌炎70例临床分析

目的:分析多发性肌炎(PM)和皮肌炎(DM)的临床特征.方法:对70例多发性肌炎和皮肌炎患者的临床资料、实验室检查、治疗及转归进行回顾性分析.结果:本组男女之比为1:1.87,发病年龄13～81岁,平均51.52岁.58例皮肌炎患者以皮肤症状为首发症状者占62%,皮肤损害中以双上眼睑紫红斑最多,占70%.乳酸脱氢酶(LDH)和肌酸磷酸激酶(CPK)阳性率分别为74.3%和51.4%,抗Jo-1抗体阳性率为1.4%.肌活检肌炎性和肌电图肌源性改变分别为90%和56.9%.恶性肿瘤伴发率约为12.9%.结论:45岁以上的患者要注意对恶性肿瘤的筛查.     

皮肌炎和多发性肌炎29例临床分析

皮肌炎和多发性肌炎２９例临床分析毛静然（浙江医科大学附属第一医院皮肤科，杭州，３１０００３）关键词：皮肌炎，多发性肌炎，临床分析我们收集１９８９－１９９４年住院治疗的皮肌炎（ＤＭ）和多发性肌炎（ＰＭ）患者共２９例，现将其临床和实验室检查分析如下。１临...     

皮肌炎与多发性肌炎63例临床分析

皮肌炎（DM）和多发性肌炎（PM）系同一自体免疫性疾病的二个类型，称为皮肌炎一多发性肌炎综合症（DM－PM）[1]。国内报道其发病率近年有上升趋势[2]。我院1989年～1995年住院治疗63例DM－PM，现分析报告如下。临床资料1．一般资料63例均为住院病人。其中DM37例，PM16例，重叠于其他结缔组织病的DM－PM9例。男25例，女38例，年龄2～65岁，平均38．4岁，其中儿童6例。冬春季发病者多，由起病至住院前病程2周～20年。有明确诱因者28例，其中感冒14例，劳累过度4例，妊娠4例，结核病及曝晒各2例，腮腺炎及外伤各1例。2．首发症状四肢…     

抗Jo-1抗体综合征1例

患者女,70岁.因咳嗽、关节痛反复发作6年,加重并出现皮损2年,于2005年8月8日入院.患者6年前无明显诱因出现持续性干咳,伴有膝关节疼痛及红肿,在外院就诊.诊断为类风湿关节炎(RA),RA肺炎,经治疔后好转.     

多发性肌炎/皮肌炎患者肠道病毒核糖核酸和中和抗体的检测及其意义

目的 探讨肠道病毒感染在多发性肌炎／皮肌炎（ＰＭ／ＤＭ）发病机理中的作用和意义。方法 用逆转录聚合酶链反应（ＲＴ／ＰＣＲ）和中和试验分别检测１６例ＰＭ／ＤＭ患者和１０例正常人对照组外周血中肠道病毒核糖核酸（ＲＮＡ）和抗体滴度。结果 １６例ＰＭ／ＤＭ患者中肠道病毒糖核酸检出率为５０。．０％,病毒中和试验中和抗体阳性率１２．５％。     

恶性肿瘤相关性皮肌炎/多发性肌炎

皮肌炎/多发性肌炎是一种原因不明的炎性肌肉疾病,常伴发恶性肿瘤,常见的为卵巢癌、乳腺癌、肺癌、胃肠道肿瘤等,而鼻咽癌在亚洲国家较常见.恶性肿瘤相关皮肌炎/多发性肌炎可能的高危因素包括高龄、男性患者、血沉增高、严重的皮损等,其发病机制可能为肿瘤细胞与肌细胞之间相似抗原的交叉免疫反应等.血清自身抗-155抗体是癌症相关性皮肌炎/多发性肌炎的血清学监测指标.     

MMP-2和MMP-9在多发性肌炎/皮肌炎患者单一核细胞中的表达

目的　探讨基质金属蛋白酶MMP-2和MMP-9 与多发性肌炎/皮肌炎（PM/DM）的关系。方法　采用实时荧光定量PCR和Western印迹方法分别检测PM/DM患者外周血单一核细胞（PMBC）中MMP-2和MMP-9的mRNA和蛋白表达水平。结果　MMP-2、MMP-9在PM/DM患者外周血PMBC中的mRNA表达水平明显高于正常人对照组,其中MMP-2为正常人对照组的2.41倍、MMP-9则为1.66倍。Western印迹检测发现MMP-2、MMP-9在PM / DM 患者外周血PMBC中的蛋白表达水平也明显高于正常人对照组,与其mRNA表达结果一致。结论　基质金属蛋白酶MMP-2和MMP-9可能在PM/DM的发生中起作用。     

皮肌炎/多发性肌炎患者血清EB病毒ZEBRA/IgG抗体的检测

皮肌炎(dermatomyositis,DM)/多发性肌炎(polymyositis, PM)是一种主要累及皮肤和肌肉的炎症性结缔组织病,常伴有关节、心肌、肺等多器官损害.研究表明,恶性肿瘤、肺纤维化和急性发病是皮肌炎患者预后的主要危险因素[1].     

皮肌炎/临床无肌病性皮肌炎患者血清中CADM-140抗体的检测和临床意义

目的 对皮肌炎（DM）/临床无肌病性皮肌炎（CADM）患者进行CADM-140抗体检测,探讨CADM-140抗体与临床特征间的联系。方法 采集38例DM（22例）/CADM（16例）患者血清,另外采集46例伴有肺间质病变的其他结缔组织病患者血清,包括8例多发性肌炎、15例系统性红斑狼疮、5例系统性硬化病、6例干燥综合征、6例混合性结缔组织病、6例特发性肺纤维化和5例正常对照者。以重组黑素瘤分化相关基因5（rMDA-5）为底物,通过ELISA检测患者血清中CADM-140抗体,比较CADM-140抗体阳性与阴性患者的临床特征。 结果 ①16例CADM和22例DM患者血清CADM-140抗体阳性例数分别为7例和2例,CADM患者阳性率（43.8%）显著高于DM（9.1%）（P ＜ 0.05）,46例伴有肺间质病变的其他结缔组织病患者及5例正常人均阴性;②CADM-140抗体阳性患者皮肤溃疡和坏死的发生率为8/9,红细胞沉降率为（40.8 ± 23.1） mm/1 h,CADM-140抗体阴性组分别为6.9%和（22.5 ± 16.8） mm/1 h,两组比较,P ＜ 0.01和 ＜ 0.05;CADM-140抗体阳性患者乳酸脱氢酶水平显著高于阴性组（分别为328.3 ± 104.2和241.1 ± 100.3 IU/L,P ＜ 0.05）,而肌酸激酶显著低于阴性组（分别为156.3 ± 260.8和1806.2 ± 3737.1 IU/L,P ＜ 0.05）;两组间抗核抗体阳性率和恶性肿瘤发生率的差异无统计学意义;③CADM-140抗体阳性患者不仅肺间质病变发生率显著高于阴性组（分别为9/9和48.3%,P ＜ 0.01）,而且急进型肺间质病变发生率也显著高于阴性组（分别为5/9和0,P ＜ 0.05）。阳性组肺高分辨率CT评分（122.9 ± 54.8）显著高于阴性组（70.0 ± 59.8）（P ＜ 0.05）。结论 通过检测CADM-140抗体不仅可以判断DM/CADM是否合并肺间质病变,还可能是伴发急进型肺间质病变的血清学标记,动态观察血清CADM-140抗体水平也许有助于预测肺间质病变病程。     

Serum concentrations of carboxyterminal propeptide of type 1 procollagen and tissue inhibitor of metalloproteinase 1 in patients with dermatomyositis

Abstract Serum levels of carboxyterminal propeptide of type I procollagen (PICP) and aminoterminal propeptide of type I procollagen (PINP) have been used as indices of collagen synthesis in patients with various fibrotic diseases during the active stages. One of the suggested contributory factors to the development of tissue fibrosis is a decrease in collagenase activity, which may be related to levels of serum tissue inhibitors of metalloproteinases-1 (TIMP-1). In this study, the serum levels of PICP and PINP in 20 patients with dermatomyositis and 29 control subjects and of TIMP-1 in 29 patients with dermatomyositis and 29 control subjects were measured using an enzyme-linked immunosorbent assay (ELISA) or a radioimmunoassay (RIA). We found that the mean PICP level in patients with dermatomyositis was significantly higher than that in normal controls (mean ± SD 326 ± 76 ng/ml vs 135 ± 88 ng/ml; P < 0.001). In 60% of dermatomyositis patients, the serum PICP level was elevated (more than 311 ng/ml, i.e. 2 × SD above the mean control value). Elevated serum PICP levels were correlated with the incidence of elevated serum creatine kinase levels in patients with dermatomyositis. The serum concentration of PINP was not elevated in comparison with that of the normal control subjects (mean ± SD 36 ± 30 ng/ml vs 63 ± 34 ng/ ml). The mean TIMP-1 level in the patients with dermatomyositis was also significantly higher than in the normal control subjects (mean ± SD 438 ± 328 ng/ml vs 163 ± 63 ng/ml; P < 0.001). In 59% of dermatomyositis patients, the mean serum TIMP-1 level was elevated (more than 289 ng/ml, i.e. 2 × SD above the mean control value). Serum PICP and TIMP-1 levels might be useful for detecting disease activity or severity in dermatomyositis. Received: 6 August 1996     

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.     

Common antigen expression between human periderm and other tissues identified by GB1-monoclonal antibody

Summary From 4 weeks estimated gestational age (EGA) until the end of the second trimester (24 weeks EGA) the fetal epidermis is covered by a specialised epithelium, the periderm. The origin and function of periderm remain speculative. We have demonstrated, using indirect immunofluorescence and immunoperoxidase staining, that periderm is recognised by a mouse IgM monoclonal antibody (Mab) GB1, which has been raised against a simple extract of human amnion. Immunoelectron microscopy localises GB1 to the amniotic surface of periderm, particularly in association with the microvilli, and also bordering cellular identations of the periderm cells. GB1 antigen (ag) is also expressed by the epithelium of fetal oesophagus, fetal and adult conjunctiva and cornea but is absent in a variety of other fetal and adult tissues including bladder, oral mucosa and thymus. The similar distribution of GB1 ag in both periderm and membranes possibly suggests a common origin and the shared expression with fetal oesophagus and fetal and adult eye may indicate a function related to the fluid environment. We therefore feel that GB1 Mab may be of use in further investigations into the origin, structure and function of human periderm.This study was presented in part at the Annual Meeting of the British Society for Investigative Dermatology, 19–20 September, 1988; ManchesterThis study was supported in part by the Dunhill Medical Trust. Collaborative work between Faculte of Medecine, Nice and Departemnt of Cell Pathology, Institute of Dermatology, London, was made possible by awards from the British Council and Glaxo Pharmaceutical to one of the authors (OMVS).     

1-2B7B: Monoclonal antibody reacting to the 120 kDa polypeptide component of human epidermal hemidesmosomes

Summary Immunohistochemical and immunochemical analyses were performed on a monoclonal antibody designated 1-2B7B which was derived from immunizing mice with human prostate epithelial tissue. The 1-2B7B antigen was expressed not only along the acinous basement membrane zone (BMZ) of the prostate and testis, but also along the BMZ of the epithelia of several other organs including the skin, oesophagus, urinary bladder, ureter, stomach, intestine and bile duct. The antigenic epitope was not expressed in these tissues of lower mammals. Immunoelectron microscopic studies on normal human skin revealed that the 1-2B7B antigen was localized mainly just beneath the hemidesmosomes of basal keratinocytes, but not beneath melanocytes. Indirect immunofluorescence and immunoelectron microscopic studies on 1 M NaCl-split skin confirmed that this antigen was not separated from the cytoplasmic membrane of basal cells after salt treatment. SDS polyacrylamide gel electrophoresis of immunochemically purified protein from the epidermis demonstrated the molecular weight of the antigen to be 120 kDa. 1-2B7B monoclonal antibody should be a useful probe for studying the pathomechanism of some blistering diseases, as well as the assembly and function of the epidermal-dermal junction.