多发性肌炎/皮肌炎自身抗体谱的研究进展

多发性肌炎/皮肌炎是一种较常见的风湿性疾病。由于其临床表现的多样性和复杂性,易造成误诊和漏诊。随着免疫学的进展,除了常用的诊断方法外,对自身抗体的检测也受到重视。多发性肌炎/皮肌炎的自身抗体主要包括3大类肌炎特异性抗体、肌炎相关性抗体和组织特异性抗体。对这些自身抗体的研究有助于疾病的诊断、分型、疾病活动情况和预后的判断等。     

皮肌炎/多发性肌炎合并ARDS患者临床特征分析

正特发性炎性肌病(idiopathic inflammatory myopathies,IIM)是一组病因未明的以四肢近端肌无力为主要表现的骨骼肌非化脓性炎症性疾病,临床以皮肌炎(dematomyositis,DM)、多发性肌炎(polymyositis,PM)多见,以肌肉和(或)皮肤损伤为主要特征。DM合并间质性肺病(interstitial lung     

抗Jo-1抗体阳性肌炎的临床与病理特点

成人炎性肌病中25%～40%血清中可查到氨酰t-RNA合成酶抗体,在这些肌炎特异性自身抗体中,第一个被发现也是最常见的是抗Jo-1抗体,其抗原是在蛋白质合成中起重要作用的组氨酰t-RNA合成酶,抗Jo-1抗体阳性的多发性肌炎（polymyositis,PM）/皮肌炎（dermatomyositis,DM）在临床上构成一组特殊症候群,称之为Jo-1综合征.该综合征在起病方式、临床表现及实验室检查方面与抗Jo-1抗体阴性的PM/DM患者明显不同.     

99例多发性肌炎和皮肌炎临床分析

目的:了解PM/DM的临床及实验室表现。方法:分析PM/DM首发症状,心血管症状、呼吸系统症状、肌酶变化等。结果:首发症状有肌无力、肌痛、呼吸困难,吞咽困难,雷诺征,关节痛或关节炎,心肌炎,发热,未梢神经炎。心血管症状发生率15·2%,呼吸系统症状发生率53·3%。结论:PM/DM是一种累及多系统的疾病,易合并恶性肿瘤。     

多发性肌炎和皮肌炎10例临床分析

多发性肌炎和皮肌炎是一组与自身免疫有关的炎症性肌病。近几年国内对本病报告渐多。现将我院从1989～1995年收治的10例报告如下。     

成人多发性肌炎/皮肌炎患者合并间质性肺病的危险因素分析与预测模型建立

目的 分析空军军医大学唐都医院风湿免疫科多发性肌炎(PM)/皮肌炎(DM)患者并发间质性肺病(ILD)的危险因素,构建临床预测模型.方法 将2015年1月1日至2021年1月1日空军军医大学唐都医院379例PM/DM住院患者纳入研究,将其分为对照组(PM/DM)和病例组(PM/DM?ILD).回顾性收集整理患者的临床病...     

特发性肌炎相关自身抗体临床应用新进展

特发性肌炎(idiopathic inflammatory myopathies, IIMs)是一组慢性、进展性、自身免疫性疾病,可累及多个组织和器官。IIMs的诊断相对困难,尤其是无肌病性肌炎,自身抗体的检测是诊断IIMs的重要参考指标,与疾病的诊断、分型、治疗和预后相关,本文就特发性肌炎病最新生物学标志物的研究进展做一综述。     

皮肌炎和多发性肌炎的护理

目的：探讨皮肌炎和多发性肌炎的护理措施。方法：回顾性分析80例皮肌炎和多发性肌炎患者的治疗效果和护理体会。结果：本组80例患者,病情好转出院72例,放弃治疗3例,死亡5例。结论：提高护理人员对疾病的认识及做好心理护理和皮肤护理,根据临床表现和并发症提供恰当、有效的护理是保证治疗效果的关键。     

皮肌炎、多发性肌炎心脏损害42例临床分析

皮肌炎、多发性肌炎 (DM,PM)主要累及横纹肌 ,心肌亦可受累 ,文献尸检报道心肌病理损害近 2 5% [1] 。为进一步提高认识 ,我们总结了 1988～ 1999年 170例DM,PM病例 ,对其中 4 2例累及心脏者进行分析。1　临床资料1.1　一般资料　 4 2例中男 17例 ,女 2 5例 ,男女比例为1∶ 1.5     

抗SRP抗体阳性和抗PM/Scl抗体阳性多发性肌炎 1例并文献复习

正多发性肌炎(polymyositis,PM)是以四肢近端肌肉受累为主要临床表现的获得性肌肉疾病,与皮肌炎、散发性包涵体肌炎及免疫介导坏死性肌病合称为特发性炎性肌病[1,2]。大多数特发性炎性肌病患者血清中可存在肌炎抗体,包括抗信号识别颗粒抗体(抗SRP抗体),有学者提出抗SRP抗体为特异     

抗SSA/Ro抗体的检测方法比较

综合比较了四种检测抗SSA/Ro抗体方法的敏感性、特异性及阳性检出率 ,得出IBT法与ID法相结合是比较实用、又能显著提高检出率的理想方法。     

Measurement of fetal atrioventricular intervals in pregnant women with anti-SSA/Ro antibodies

Journal of Medical Ultrasonics - The objective of our study was to compare and consider reference values of fetal atrioventricular (AV) intervals as measured by four different pulsed Doppler wave...     

皮肌炎患者抗核抗体和抗Jo-1抗体检测的临床意义

目的探讨皮肌炎常见自身抗体抗核抗体(ANA)和抗合成酶Jo-1抗体(抗Jo-1抗体)的临床意义。方法选取皮肌炎患者280例,分为ANA阳性且抗Jo-1抗体阳性组(A组,70例)、ANA阳性抗Jo-1抗体阴性组(B组,80例)、ANA阴性抗Jo-1抗体阳性组(C组,70例)及ANA阴性且抗Jo-1抗体阴性组(D组,60例)。分析4组患者肌酶变化情况、内脏器官损害情况及实验室相关指标特点,并分析不同ANA水平与肌酶的相关性。结果与D组比较,A组和C组患者肌酶明显升高,且肺和心脏损害发生率也明显升高(P0.05),而B组患者肌酶升高不明显(P0.05),且肌酶与ANA滴度水平变化无相关性(P0.05)。结论抗Jo-1抗体能正确反映皮肌炎病情严重程度。     

母体抗SSA/SSB抗体阳性胎儿完全性房室传导阻滞超声表现

目的 观察母体抗SSA/SSB抗体阳性胎儿完全性房室传导阻滞(CAVB)超声表现.方法 以二维、M型及多普勒超声技术观察7胎母体抗SSA/SSB抗体阳性CAVB胎儿心脏,获取二尖瓣、左心室流入道及流出道、脐动脉(UA)、静脉导管(DV)及大脑中动脉(MCA)血流频谱.结果 7名孕妇抗SSA抗体、抗Ro52抗体均呈阳性,...     

Acute respiratory distress syndrome in a polymyositis patient with the anti-Jo-1 antibody

We report a case of a 66-year-old caucasian woman suffering from polymyositis with the anti-Jo-1 antibody. Shortly after admission to our hospital, despite the fact that the patient was given intravenous pulse methylprednisolone (1000 mg/day for 3 consecutive days), she developed severe, ventilatory-dependent, acute respiratory distress syndrome. Evaluation for infectious or noninfectious aetiologies of acute respiratory distress syndrome was unrevealing. Bronchoalveolar lavage disclosed neutrophilic alveolitis. Histological examination of a transbronchial biopsy revealed an interstitial fibrosing process and cuboidalisation of the alveolar epithelium. In addition to high-dose methylprednisolone the patient was given intravenous pulse cyclophosphamide fortnightly for six weeks and afterwards every 4 weeks. All symptoms resolved and arterial blood gases returned to normal. Remission has been maintained with azathioprine. One year after onset, polymyositis is in complete remission. This is the first report of a patient suffering from polymyositis with the anti-Jo-1 antibody who survived such a complication. Intravenous pulse cyclophosphamide was probably a life saving therapy.     

皮肌炎/多发性肌炎死亡危险因素分析

目的 回顾性总结皮肌炎（DM）或多发性肌炎（PM）患者的临床特征,分析DM/PM死亡的独立危险因素。方法 回顾性分析2016年1月~2019年9月在河北医科大学第二医院初次就诊并符合2004年国际肌病协作组建议的特发性炎症性肌病（IIM）分类诊断标准的212例DM/PM患者的临床资料, 根据半年内是否死亡分为研究组（死亡组）和对照组（非死亡组）,分析两组临床特点及实验室检查的差异,总结DM/PM死亡的独立危险因素。结果 两组间男性、白蛋白（ALB）<35 g/L、铁蛋白升高、抗MDA5抗体阳性、肺部感染、间质性肺疾病差异有统计学意义（P<0.05）;Logistic回归分析ALB<35 g/L、铁蛋白升高、抗MDA5抗体阳性、肺部感染、间质性肺疾病与DM/PM死亡密切相关（P<0.05）。结论 男性、ALB<35 g/L、铁蛋白升高、抗MDA5抗体阳性、肺部感染、间质性肺疾病为DM/PM死亡的独立危险因素,提示预后不良。     

Impaired clearance of apoptotic cardiocytes is linked to anti-SSA/Ro and -SSB/La antibodies in the pathogenesis of congenital heart block

The role of cardiocytes in physiologic removal of apoptotic cells and the subsequent effect of surface binding by anti-SSA/Ro and -SSB/La antibodies was addressed. Initial experiments evaluated induction of apoptosis by extrinsic and intrinsic pathways. Nuclear injury and the translocation of SSA/Ro and SSB/La antigens to the fetal cardiocyte plasma membrane were common downstream events of Fas and TNF receptor ligation, requiring caspase activation. As assessed by phase-contrast and confirmed by confocal microscopy, coculturing of healthy cardiocytes with cardiocytes rendered apoptotic via extrinsic pathways revealed a clearance mechanism that to our knowledge has not previously been described. Cultured fetal cardiocytes expressed phosphatidylserine receptors (PSRs), as did cardiac tissue from a fetus with congenital heart block (CHB) and an age-matched control. Phagocytic uptake was blocked by anti-PSR antibodies and was significantly inhibited following preincubation of apoptotic cardiocytes with chicken and murine anti-SSA/Ro and -SSB/La antibodies, with IgG from an anti-SSA/Ro- and -SSB/La-positive mother of a CHB child, but not with anti-HLA class I antibody. In a murine model, anti-Ro60 bound and inhibited uptake of apoptotic cardiocytes from wild-type but not Ro60-knockout mice. Our results suggest that resident cardiocytes participate in physiologic clearance of apoptotic cardiocytes but that clearance is inhibited by opsonization via maternal autoantibodies, resulting in accumulation of apoptotic cells, promoting inflammation and subsequent scarring.     

多发性肌炎/皮肌炎患者血清中外泌体的分离鉴定

目的从多发性肌炎/皮肌炎(polymyositis/dermatomyositis,PM/DM)患者外周血血清中分离鉴定外泌体(exosome),并进行初步的蛋白质研究。方法用Exo QuickTM试剂盒分离纯化PM/DM患者血清exosome;透射电镜观察其形态特征,Nanosight可视型纳米颗粒分析仪检测粒径大小分布情况;western blot鉴定exosome的表面标志物CD9,CD81,Flotillin-2;BCA法对其所携带的蛋白质进行定量分析;十二烷基硫酸钠聚丙烯酰胺凝胶电泳(SDS-PAGE)初步分析其蛋白质组分。结果透射电镜下患者血清exosome呈圆形或椭圆形膜性小囊泡,直径分布范围92±67 nm;western blot结果表明,PM/DM血清来源的exosome存在表面标志物CD9、CD81、Flotillin-2;BCA法测定PM/DM患者exosome总蛋白浓度为14.68(6.00,32.55)μg/μL,健康组exosome总蛋白浓度为14.09(8.00,23.28)μg/μL,SDS-PAGE显示两组血清exosome均在Mr(×103)为55~70处有高丰度蛋白质富集,在Mr(×10~3)为40~55处存在差异条带。结论成功从PM/DM患者的外周血中分离出血清exosome,并为寻找差异蛋白质提供实验依据。