混合结缔组织病三例

结缔组织病是一种免疫复合体病,它包括系统性红斑狼疮(SLE),系统性硬皮病(PSS),皮肌炎(DM)或多发性肌炎(PM),类风湿性关节炎及风湿热,结节性动脉周围炎。此外,也有人将白塞氏综合征,韦格氏肉芽肿,干燥综合征等归入结缔组织病的近缘疾病。1972年 Sharp氏将临床上表现有类似 SLE,PSS 及DM 各种症状混合存在,但无肾脏损害,荧光抗体检查为高滴度斑点型,对皮质类固醇激素治疗反应良好的一种病,命名为混合结缔组织病(MCTD)。由于 MCTD     

抗D'E多肽抗体在混合结缔组织病中的应用分析

混合结缔组织病(MCTD)是一组综合征,以系统性红斑狼疮(SLE),进行性系统性硬化症(PSS)和多发性肌炎/皮肌炎(PM/DM)的混合表现为特点,兼有滴度很高的抗rRNP抗原的抗体[1],对MCTD的诊断国际上尚无统一的标准.由于在SLE,PSS,PM/DM等多种自身免疫性疾病中都可找到对诊断有高度特异性的自身抗体.若能在MCTD中找到这类特异性自身抗体,有助于对该病的诊断.     

多发性肌炎/皮肌炎134例临床分析

目的:探讨多发性肌炎(PM)和皮肌炎(DM)患者的临床特点,以利临床诊断、指导治疗及判断预后.方法:回顾性分析本院134例PM和DM患者的临床首发症状及病程中临床表现、肌酶水平、肌电图、肌活检、治疗以及转归,并对PM和DM进行分析比较.结果:PM首发症状以发热肌痛、近端肌无力多见,DM则不论其病程长短均多数以皮肤损害为首发症状.PM患者出现肌酶升高明显高于DM组患者,CK在PM组和DM组中治疗前后的改变具统计学意义(P＜0.05).PM的病理改变主要表现为散在肌纤维萎缩;DM以束周萎缩多见.影响预后的主要因素年龄、肺部感染有关.结论:多发性肌炎和皮肌炎的临床特征及病理各有不同.     

多发性肌炎、皮肌炎中医辨治临床研究

多发性肌炎(polymyositis,PM)、皮肌炎(dermatomyositis,DM)是横纹肌非化脓性炎性肌病,主要表现为对称性的肌肉无力、肌肉疼痛,尤其是肢体近端肌肉受累明显,肌酶明显增高,皮肌炎病人尚合并皮肤损害.     

血管因素在皮肌炎和多发性肌炎发病中的作用

多发性肌炎(PM)和皮肌炎(DM)是一组免疫介导的自身免疫疾病,临床主要表现为对称性肢体近端无力.DM患者还有皮肤受累症状.肌肉活检多表现为肌纤维坏死、再生和单核细胞浸润[1].近年来研究认为,肌内血管病变是原发损害,在疾病的发生、发展中起重要作用.血管病变在多发性肌炎患者则不明显,提示二者的发病机制可能不同.     

76例多发性肌炎临床分析

为探讨多发性肌炎患者心脏损害的改变，对76例多发性肌炎患者临床资料进行分析。结果，33例多发性肌炎患者有心脏损害的表现，经超声心动图(UCG)、心电图(ECG)检查异常率分别为33％、43．4％，而且多发性肌炎患者心脏瓣膜病变表现单或多瓣膜受累。结论多发性肌炎患者心脏损害并不少见，但是表现不具有特异性，UCG、ECG的检查方法有助于早期诊断多发性肌炎的心脏损害。     

35例多发性肌炎临床肌电图及肌活检分析

目的通过对35例多发性肌炎(PM)临床资料分析,探讨其临床特点。方法采用回顾性研究的方法。结果35例PM患者血清酶学增高,肌电图示肌源性损害,肌活检发现肌纤维坏死和炎性细胞浸润。结论肌电图、肌活检对多发性肌炎的诊断有重要临床意义。     

多发性肌炎１７例临床及随访分析

目的　总结多发性肌炎(PM)的临床特点及随访了解本病转归.方法　分析１７例PM 患者的临床及随访资料并复习文献.结果　１７例患者的平均发病 年龄(４０±２５)岁,男:女为１:１．８.以亚急性起病者居多,首发症状以四肢无力、肌痛多见.３例抗Jo－１抗体阳性患者均合并肺损害.９４．１％的患者血清CK 升高; １５例肌电图呈肌源性损害,１例表现为神经源性合并肌源性损害,１例表现为神经源性损害;１０例患者行双下肢肌肉MRI检查,其中９０％有异常信号;肌肉病理检 查１００％呈肌源性损害.用糖皮质激素、免疫抑制剂、丙种球蛋白等药物联合治疗后,８８．２％的患者预后良好.随访１－５年,４例复发,再次治疗有效,２例合并恶性 肿瘤死亡.结论　PM 多以亚急性起病为主,发病率女多于男,首发症状多为四肢无力、肌痛.抗Jo－１抗体阳性提示可能伴有肺部损害;结合血清肌酶、肌电图、肌 肉MRI、肌肉活检等诊断技术可提高PM 临床诊断水平,多种治疗方法联合使用提高疗效,除合并恶性肿瘤,预后良好.     

以心脏损害为首发表现的多发性肌炎/皮肌炎七例临床分析

目的总结以心脏损害为首发表现的多发性肌炎/皮肌炎的心脏损害特点.方法收集423例多发性肌炎/皮肌炎患者的临床资料,分析其中7例以心脏损害为首发表现的多发性肌炎(6例)、皮肌炎(1例)的心脏损害情况.结果7例以心脏损害为首发表现者心脏受累严重,表现复杂多样,且均为联合损害.结论以心脏损害为首发表现的多发性肌炎或皮肌炎非常少见,但心脏损害可作为多发性肌炎或皮肌炎的首发表现,应引起临床医师的注意.     

混合性结缔组织病两例报告

混合性结缔组织病(MCTD)是一组病因不甚明瞭的胶原-血管性疾病,它包括风湿热(RF)类风湿性关节炎(RA)、系统性红斑狼疮(SLE)、皮肌炎(DM)、多发性肌炎(PM)、进行性硬化症(PSS)、结节性多动脉炎(PAN)、强直性脊椎炎(AS)及尿道-关节-结膜综合征等。由于病变累及多系统,故临床表现错综复杂且重叠交错,其病程大都呈亚急性或慢性经过。因此争取早期诊断,合理治疗,对减轻或防止脏器的损害、控制病情或预后转归等都有很大关系。我科近期内有两例儿童MCTD现报道如下:     

Overlap syndrome of progressive systemic sclerosis and polymyositis: report of 40 cases.

Forty cases of overlap syndrome of progressive systemic sclerosis and polymyositis (OS PSS-PM) are reported in this paper. All of these cases had manifestations of both PSS and PM as well as Raynaud's phenomenon. The sclerodermatous skin changes were diffused over the whole body in most cases. All cases had muscular weakness, elevated skeletal muscle enzyme levels and muscle damage as seen on the electromyogram. Histopathologic changes showed characteristics of myositis. There was noticeable systemic involvement, especially with the digestive and circulatory systems. Serologic examination frequently revealed autoantibodies. The patients responded well to traditional Chinese medicines and corticosteroids.     

磁共振扩散张量成像定量分析在评价多发性肌炎和皮肌炎临床特征的应用

目的分析磁共振扩散张量成像定量(DTI)分析在评价多发性肌炎和皮肌炎(PM/DM)临床特征的应用。方法将本院自2017年3月-2019年3月收治的200例活动期PM/DM患者纳入PM/DM组,另筛选同期30例健康志愿者为对照组,所纳入患者均有双侧大腿DTI扫描结果,比较大腿肌肉前群、内群、后群肌肉组织中水肿肌肉、对照组(正常肌肉)、未受累肌肉ROI区域的平均扩散系数(ADC)、各向异性分数值(Fractional anisotropy,FA值)、本征值(λ1、λ2、λ3)。结果①各组不同肌肉组织A D C值比较差异有统计学意义(P<0.05);对照组、PM/DM水肿肌肉、PM/DM未受累肌肉均以VI处ADC值最高,对照组、PM/DM未受累肌肉以GA处ADC值最低,PM/DM水肿肌肉以SM处ADC值最低;组间比较PM/DM水肿肌肉A DC值均显著高于PM/DM未受累肌肉、对照组(P<0.05);②各组不同肌肉组织FA值比较差异有统计学意义(P<0.05),对照组、PM/DM水肿肌肉、PM/DM未受累肌肉均以ST FA值最高,AD FA值最低;但组间FA值比较差异均无统计学意义(P> 0.05);③各组大腿肌肉λ1均值>λ2>λ3 (P <0.05);组间比较PM/DM水肿肌肉λ1、λ2、λ3均值均显著高于PM/DM未受累肌肉、对照组(P<0.05)。结论 DTI或可基于PM/DM患者水肿肌肉的各向异性特征对病变肌肉的病理变化进行定量分析,值得临床重视。     

Clinical and serologic features of patients with polymyositis or dermatomyositis

The clinical and serologic features of 36 patients with polymyositis (PM) or dermatomyositis (DM) were observed over a 5-year period. The mean age of the patients at the time of diagnosis was 48.5 years, and 61% were female. According to widely accepted diagnostic criteria 50% had PM (group I), 14% DM (group II), 11% PM or DM associated with malignant disease (group III) and 25% PM or DM associated with a connective tissue disorder (group V). None of the patients had childhood PM or DM associated with vasculitis (group IV). All the patients had muscle weakness, and 94% of the patients tested had an elevated serum level of creatine kinase. The average delay from the onset of symptoms to diagnosis was 14 months overall but only 2.3 months for the DM patients. Of the 30 patients whose serum was tested, 73% had antinuclear antibodies, with antibodies to nuclear ribonucleoprotein being most common in group V patients and antibodies directed against the Jo-1 antigen being restricted to patients with PM alone (group I).     

化脓性肌炎8例的MR诊断

目的分析化脓性肌炎的MR表现特点,探讨MR对化脓性肌炎的诊断价值。方法回顾分析经手术及病理证实的8例化脓性肌炎的MR表现并进行总结。结果 8例MR显示受累肌肉弥漫性肿大,T2WI上表现为较明显不均匀高信号,STIR上为明显高信号;而TlWI上呈与邻近肌肉等、略低或略高信号改变,邻近肌间隙水肿。4例病灶中见单个或多个TlWI低信号、T2WI高信号脓肿形成,周围脓肿壁在T1WI上为相对高信号。增强扫描受累肌肉呈明显弥漫性强化2例,2例脓肿形成者呈环状强化,脓腔及小的炎性坏死区无强化。结论 MR可清晰显示化脓性肌炎病变部位、特点及范围,具有重要的诊断价值。     

脊柱结核的MRI表现

目的探讨脊柱结核的MRj表现,分析其特征。方法回顾分析44例经手术病理证实的脊柱结核MRI征象,观察榷体、终板、椎间盘、冷脓疡的信号变化和增强后改变。结果（1）44例共有110个椎体受累,单椎体2例,2个椎体30例,3个及以上椎体12例;椎体破坏于T1WI呈片状均匀低信号、混杂信号,T2WI及T2脂肪抑制序列呈均匀高信号、混杂信号;终板局限破坏,见囊状小脓肿形成,呈无结构长T1、长T2信号。（2）39例为单个椎间盘受累,其中轻度变窄20例,中至重度变窄19例,T2WI呈低信号,T2wI及T2＋STIR呈不均匀高信号,椎间盘正常者5例。（3）44例椎旁软组织肿胀,椎前或椎旁脓肿39例,其中,10例伴腰大肌脓肿,4例伴椎管内脓肿。脓肿呈T2WI稍低、T2WI及T2＋STIR明显高信号。增强扫描,脓肿壁呈不均匀条带状或环形强化。（4）15例患者21个椎弓根受累,18例脊髓受压。结论椎体及椎间盘破坏、椎体破坏伴椎旁脓肿为脊柱结核特征,MRj能发现脊柱结核早期征象,反映其病理变化。     

原发性干燥综合征合并肌炎患者临床特征分析

分析原发性干燥综合征（pSS）合并肌炎患者的临床特点,并与原发性多发性肌炎（PM）患者比较。结果示202例pSS患者中有4例（2．0％）在诊断pSS后5—20年出现肌炎表现,其中2例无明显肌痛和肌无力症状;肌酸肌酶（CK）最高值为（480—2702）IU／L,中位数为789．5IU／L,抗Jo-抗体均阴性;糖皮质激素治疗1周后CK下降13．9％～71．5％,中位数为48．9％;随访8个月至5年,无一例肌炎病情反复。与15例PM患者资料比较分析,两组在肌痛发生率、CK最高值、激素治疗后CK下降率、抗Jo-1抗体阳性率等方面差异有统计学意义。提示pSS患者合并肌炎较为少见,多在干燥综合征多年之后出现,肌痛和肌无力表现较轻,使用激素效果较好。     

二维斑点追踪显像技术评价慢性缺血性二尖瓣返流患者乳头肌功能的超声研究

目的本研究应用二维斑点追踪显像技术测量慢性缺血性二尖瓣返流(IMR)患者乳头肌收缩运动的二维应变及应变率,以探讨此技术对评价慢性IMR患者乳头肌功能的临床应用价值。方法纳入陈旧性心肌梗死且合并二尖瓣返流的患者119例,正常对照组94例。测量左室两组乳头肌的收缩期峰值应变(PSS)和应变率(PSSR)。根据病例组患者心肌梗死部位不同又分为前壁(AMI)和下壁(IMI)心肌梗死两个亚组,比较不同亚组上述参数间的差异以及与二尖瓣返流程度的关系。结果慢性IMR组的两组乳头肌PSS、PSSR均较正常对照组降低;IMI组前、后乳头肌的PSS、PSSR均较AMI组低;IMI组的二尖瓣返流以中、重度为主,而AMI组则以轻、中度为主。结论二维斑点追踪显像技术可较准确评价慢性IMR患者乳头肌的运动功能,为进一步的临床研究提供了无创、可信赖的新方法。     

多发性肌炎和皮肌炎38例的临床与肌电图分析

目的 探讨多发性肌炎（PM）和皮肌炎（DM）患者的临床与肌电图特征.方法 对38例PM和DM患者的临床与肌电图的特征进行回顾分析.结果 临床症状以肌肉无力最为多见（88.0%）,其他依次为皮肤损害（31.0%）,发热（10.5%）.肌电图检查示肌源性损害32例,神经源性损害1例,异常率为86.8%.主要表现为插入电位延长（9.5%）、出现自发电位（52.3%）;MUP时限缩短（74.6%）、MUP波幅降低（6.3%）,多相波增多（34.1%）;重收缩时波型异常（44.4%）及峰值波幅降低（37.3%）.股四头肌、胫前肌及胸锁乳突肌阳性率显著高于拇短展肌组.胫前肌组MUP时限缩短的阳性率及多相波增多阳性率均表现最高.结论 肌电图检查是诊断PM和DM的重要手段,选择胸锁乳突肌、胫前肌及股四头肌进行EMG检查阳性率最高.     

皮肌炎/多肌炎表观遗传学标志物的研究进展

Idiopathic inflammatory myopathy (IIM) is a rare group of autoimmune diseases, characte-rized by chronic muscle weakness, muscle fatigue and infiltration of single nuclear cells in skeletal muscle. Its subtypes include dermatomyositis (DM), polymyositis (PM), inclusion body myositis (IBM) and immune-mediated necrotizing myositis (IMNM), and the most common subtypes are DM and PM. PM is an autoimmune disease mainly manifested by muscle damage. When the skin is involved, it is called DM. The incidence of IIM was relatively low, which was 1.16-19 per million people/year, but the mortality was high and the prognosis was poor. The pathogenesis of IIM is still unclear. Previous stu-dies suggest that both immune and non-immune mechanisms are involved in its pathogenesis, especially cellular and humoral immunity. In recent years, researchers have conducted a number of studies on the pathogenesis of IIM, especially in the study of DM/PM with the application of high-throughput biome-trics. Epigenetics is a discipline that refers to the genetic phenomena of DNA methylation spectrum, chromatin structure state and gene expression spectrum transferred between cells without any changes in DNA sequence, including DNA methylation, chromatin modification and non-coding RNA changes. A large number of studies have shown that epigenetic modification plays an important role in many diseases, especially in cancer. Recent studies have also found a series of epigenetic markers related to the occurrence and development of DM/PM, mainly in the aspect of non-coding RNA changes, such as miR-10a, miR-206, etc.. And there has also been some research on DNA methylation. However, no studies have been reported on whether chromatin modification is involved in the pathogenesis of DM/PM. The pathogenesis of DM/PM is complex and diverse. With the development of research, certain microRNAs (miRNAs) and long non-coding RNAs (lncRNAs) may become biological markers for the early diagnosis of DM/PM. Therefore, this paper mainly expounds the research progress of the biomarkers of DM/PM from the aspect of epigenetics.