多发性肌炎、皮肌炎诊断治疗中的几个问题

多发性肌炎(polymyositis，PM)、皮肌炎(dermatomyositis，DM)是一组免疫性炎性肌病，其共同表现为对称性四肢近端肌肉及延髓支配肌肉力弱，血清肌酸激酶(CK)升高，肌电图呈肌源性改变，肌肉病理表现为肌纤维坏死、再生和炎性细胞浸润。近年来已经对其临床表现多样性的认识，发病机制的了解，以及治疗取得了一些进展，但是对发病机制尚未完全阐明，对各项诊断技术的评价尚未标准化，对治疗规律缺乏精确了解，对繁杂的治疗方法尚需规范。因此，有必要对一些问题进行深入的研究、讨论。     

多发性肌炎与皮肌炎

本病是较多见的肌肉疾病,属自体免疫性疾患。临床特点是肢体近端肌无力及萎缩。病理特点是肌肉有炎症浸润和肌纤维坏死。激素或免疫抑制剂治疗部分病例有效。所谓肌炎,以往既指原因已明的(如感染),也指原因不明的肌炎,现今则指病因不明或与免疫有关的肌炎。     

多发性肌炎与皮肌炎的临床病理研究

目的 探讨多发性肌炎和皮肌炎的病理变化、发病机制、治疗效果和预后。方法 按照Bohan的多发性肌炎和皮肌炎的诊断分类标准,将本研究中的58例多发性肌炎和皮肌炎患者分成4类亚型。对全部患者进行肌肉病例及血甭酶学检查,并进行治疗观察。结果 临床表现以肌无力、肌肉压血清酶谱增高、肌电图及病理学异常等为特征。但各型之间又有差异,提示存在不同的发病机制,治疗以激素为主,必要时加用免疫抑制剂。结论 预后治疗时机密切相关,症状随血清酶谱下降而了转,肺部感染、心肌病损及病程迁延提示预后不良。     

多发性肌炎/皮肌炎免疫发病机制研究进展

多发性肌炎/皮肌炎病因未明,目前多认为与免疫相关。本文从细胞毒性T细胞、TCR基因重排、主要组织相容性复合体、细胞因子、趋化因子和黏附分子、凋亡、自身抗体以及基质金属蛋白酶等不同方面就其免疫发病机制进行综述。     

多发性肌炎/皮肌炎肌肉组织浸润的炎细胞亚群分析

目的:探讨多发性肌炎(PM)、皮肌炎(DM)和重迭综合征(IM+)骨骼肌组织损伤的免疫机制。方法:应用抗单个核细胞(MNC)亚群的系列单克隆抗体进行免疫组织化学LSAB法染色,分析31例PM/DM患者肌活检组织MNC亚群的定位和分布。结果:肌活检标本中PM组浸润的炎细胞主要位于肌内膜,其次为肌束膜,以CD3+T细胞和巨噬细胞为主,并可见CD8+T细胞和巨噬细胞侵入肌纤维;IM+组炎细胞主要位于肌束膜,巨噬细胞最多,其次为CD3+T细胞;DM组炎细胞以血管周围为主,主要为巨噬细胞。定量分析每高倍视野的CD68+细胞数PM和IM+相比P<0.05,有显著性差异。结论:体液免疫和细胞免疫在PM、DM和IM+发病过程中均起重要作用。     

多发性肌炎与皮肌炎（附20例临床分析）

目的 探讨多发性肌炎与皮肌炎的临床特点。方法 回顾性地分析了20例多发性肌炎与皮肌炎患者的临床资料,并结合文献复习其肌电图表现,病理特点,肌酶变化特点及治疗进展。结果 20例均表现出不同程度和不同部位的肌无力和肌萎缩。部分病例伴有皮肤损害和其他系统的损害。大部分病例肌酶均明显升高,肌电图以肌源性损害为主,异常率达95%,6例肌肉活检的病理表现出特征性的改变。结论 多发性肌炎和皮肌炎是一组以肌无力和肌萎缩为主要表现的自身免疫性疾病,本病的诊断应结合临床资料,肌酶和肌电图检查,有条件应行肌肉活检进行确诊,同时应进行全身各系统的全面检查寻找可能存在的恶性疾病。治疗可用激素,免疫抑制剂等,也可试用免疫球蛋白和甲基强地松龙冲击治疗。     

难治性多发性肌炎／皮肌炎的治疗进展

难治性多发性肌炎／皮肌炎的治疗进展焉传祝综述李大年审校类固醇激素是治疗多发性肌炎／皮肌炎（ＰＭ／ＤＭ）的首选药物，但约有２０％的病人对类固醇治疗无效或不能耐受，称为难治性（ｉｎｔｒａｃｔａｂｌｅ）ＰＭ／ＤＭ，其临床处理比较困难。近年来，人们对难治性Ｐ...     

多发性肌炎与皮肌炎49例临床分析

多发性肌炎是一组以许多骨骼肌的间质性炎变和肌纤维变性为特征的综合征。一部分伴有皮肤的炎变,称皮肌炎。目前,多认为本病是一种自身免疫性疾病。我们自1981～1990年共收治49例,现报告如下。     

多发性肌炎和皮肌炎细胞免疫的特点

PM和DM的病因目前仍不清楚,但病因方面的一些线索已经出现,发病的大致过程如下:诱发因素现仍不清楚,可能是病毒,这些来自病毒的抗原和/或肌自身抗原的多肽由巨噬细胞提呈给T细胞,导致T淋巴细胞增殖分化,活化的T细胞释放IL-2和IFN-r等细胞因子。IL-2能促进T细胞进一步活化。而IFN-r能激活吞噬细胞,后者释放TNF和IL-1。局部释放的TNF和IFN-r诱导肌细胞表达MHC-1,Ⅱ类抗原和ICAM-1。MHC-Ⅱ类分子提呈抗原刺激CD4~+的T细胞。而ICAM-1能促进单核细胞进一步向病灶部位趋化。最后B淋巴细胞产生抗体。CD8~+的T细胞直接或释放淋巴因子导致肌损害。而吞噬细胞直接或释放TNF进一步加速肌损害,现在人们需进一步研究确定是哪些诱发因素与PM,DM有关,以及如何来阻止免疫介导的肌损害的发生。     

多发性肌炎、皮肌炎酶组织化学、组织化学诊断探讨

多发性肌炎、皮肌炎 (PM/DM)是一组与自身免疫有关的炎症性肌病。我们采用先进的组织化学和酶组织化学技术对 82例 PM、 DM患者的肌肉进行回顾性研究 ,以探讨组织化学和酶组织化学技术在病理诊断中的意义。材　料　和　方　法1.多发性肌炎 (PM) :女 34例 ,男 10例 ;最小17岁 ,最大 6 3岁 ,平均 30 .5岁 ,以中年为最多见。平均就诊时间 1年 4个月 ,最长病程 7年 ,最短 7天。近端肌力 、 级为最多见 ,偶见肌力为 级。其中吞咽困难占 31% ,肌痛占 5 0 % ,血沉快占 18% ,肌无力 10 0 % ,发热 35 % ,关节痛 13% ,雷诺氏征 4 .3% ,心肌酶升…     

炎症性肌病中磁共振成像的诊断价值

目的探讨炎性肌病中磁共振成像的表现及其诊断价值。方法对16例多发性肌炎、3例包涵体肌炎行双下肢磁共振成像SE序列T1WI,快速自旋回波序列(FSE)T2WI扫描。结果多发性肌炎与包涵体肌炎均表现为:在T1WI上为等T1信号,在快速自旋回波序列T2WI上表现为两侧对称性、小片状分布的稍高信号,肌束形态无明显异常,肌束界限清楚。结论磁共振成像是诊断炎性肌病有效的辅助方法。自旋回波序列(FSE)T2WI是诊断肌炎最敏感的序列,对肌炎肌肉活检的部位的选择有指导意义。     

单纯性多发性肌炎和皮肌炎的病程演变

目的　探讨单纯性多发性肌炎和皮肌炎的病程演变规律,以期获得对该病较全面的认识,指导临床治疗。方法　对６２ 例单纯性多发性肌炎和皮肌炎病程演变、临床病理和治疗相互间关系进行分析研究。结果　起病急、进展快、病情在半年内发展至高峰者,临床上以发热、肌痛、颈肌无力和吞咽困难常见,病理以急性炎症改变突出,及时治疗疗效好;而起病隐匿、进展缓慢、病情半年以上仍在进展者,以肢体肌无力多见,病理以间质改变更突出,预后较差,易迁延。结论　我们认为,病情在半年以内达高峰者可称为急性或亚急性单纯性多发性肌炎或皮肌炎,病情超过半年仍在进展者可称为慢性多发性肌炎或皮肌炎。     

Magnetic resonance imaging of the muscles in patients with polymyositis and dermatomyositis

Magnetic resonance imaging (MRI) of the muscles was performed in patients with polymyositis and dermatomyositis. Lesions with high intensity on T2-weighted image, but normal intensity on T1-weighted image, were observed in 7 of 8 patients in the active stage of the disease. Following clinical improvement with corticosteroid therapy in 4 patients, the high intensity lesions reverted to normal. The high intensity lesions seen on T2-weighted image in the active stage may represent edema and inflammation of the muscle. MRI of the muscle may serve as a diagnostic tool and be useful for follow-up of the patients with polymyositis or dermatomyositis.     

皮肌炎和多发性肌炎175例神经传导检测

目的 研究皮肌炎(dermatomyositis,DM)和多发性肌炎(polymyositis,PM)合并神经传导检测(NCS)异常患者的临床和电生理特点以及病因探讨.方法 收集2005年1月到2008年9月中国医学科学院北京协和医院病房收治的DM或PM确诊病例175例,对其临床和NCS结果进行回顾性分析.结果 175例患者中,NCS异常者66例,其中明确的周围神经病32例(48.5%),不能肯定为周围神经损害者34例(51.5%).合并周围神经损害的DM或PM患者恶性肿瘤(3/32,9.3%)、其他免疫病(6/32,18.8%)的发生率较无周围神经损害的DM或PM患者(4/109,3.7%;7/109,6.4%)有明显增加(X2=13.653,P=0.003).结论 合并周围神经损害的DM或PM患者恶性肿瘤、其他免疫病的发生率明显增加,NCS可以为临床早期诊断提供更多的提示和帮助.     

Magnetic resonance imaging of skeletal muscles in the polymyositis

Magnetic resonance imaging of skeletal muscles was performed in 11 patients with polymyositis. Two types of muscle lesions were revealed. The first, inflammation, showed increased signal intensity on T2-weighted images and iso-intensity on T1-weighted images. The second, fatty replacement, showed increased signal intensity on both images. The coronal sections could elucidate the extension of the lesion in each affected muscle. Inflammation was relatively diffuse, while homogeneous fatty replacement tended to begin at the lower myotendinous junctions. © 1997 John Wiley & Sons, Inc. Muscle Nerve 20: 1463–1466, 1997     

Magnetic resonance imaging for diagnosis of early Alzheimer's disease

A major challenge for neuroimaging is to contribute to the early diagnosis of Alzheimer's disease (AD). In particular, magnetic resonance imaging (MRI) allows detecting different types of structural and functional abnormalities at an early stage of the disease. Anatomical MRI is the most widely used technique and provides local and global measures of atrophy. The recent diagnostic criteria of “mild cognitive impairment due to AD” include hippocampal atrophy, which is considered a marker of neuronal injury. Advanced image analysis techniques generate automatic and reproducible measures both in the hippocampus and throughout the whole brain. Recent modalities such as diffusion-tensor imaging and resting-state functional MRI provide additional measures that could contribute to the early diagnosis but require further validation.     

神经梅毒26例磁共振成像表现

目的 分析不同临床类型神经梅毒的MRI表现.方法 回顾性研究26例神经梅毒患者的临床及MRI资料,描述各种临床类型神经梅毒的MRI表现.结果 26例神经梅毒患者中,17例MRI有异常表现.其中脑膜血管型梅毒7例,主要表现为脑部多发的缺血灶、梗死灶,少数表现为脑炎样改变;麻痹性痴呆6例,主要表现为额、颞叶萎缩,少数伴有脑缺血灶、颗粒性室管膜炎及海马硬化;脊髓膜血管梅毒3例,主要表现为下颈段至下胸段脊髓轻度肿胀,其内可见多发的缺血灶;脊髓痨1例,其脑部MRI表现为缺血灶.9例患者MRI表现正常,其中脑膜型梅毒4例,脊髓痨5例.结论 不同临床类型神经梅毒的MRI表现具有一定特征,但缺乏特异性,临床上容易误诊.     

Magnetic resonance imaging of dysraphic myelodysplasia

The spinal cord in 56 children and adolescents was examined by magnetic resonance imaging (MRI) many years after neonatal surgery on a meningomyelocele (average age 12 years). In a high percentage of cases, the diagnosis tethered cord was made. Associated anomalies were found with a frequency of 21%. Typical findings are presented and the impact of these results on therapy planning is discussed.     

Magnetic resonance imaging and ultrasonography in diagnosis of pelvic vein thrombosis during pregnancy

Introduction

Pelvic deep vein thrombosis (DVT) is difficult to diagnose during pregnancy. In a two-center trial, we evaluated the agreement between ultrasonography and magnetic resonance imaging (MRI) in diagnosing the extent of DVT into the pelvic veins during pregnancy.

Materials and methods

Pregnant women with proximal DVT were examined both with ultrasound and MRI as part of a study designed for treatment of DVT during pregnancy. Ultrasound was performed using color flow by specialist in vascular ultrasound with Doppler and compression techniques. The MRI sequences consisted of a 2D Time of Flight angiography with arterial flow suppression and maximum intensity projection reconstructions; a 3D, T1-w-prepared gradient echo sequence with fat saturation for thrombus imaging; a steady-state free precession sequence; and a Turbo-Spin-Echo. No contrast agent was used. Proportion of agreement (κ) for detection of DVT in individual veins was measured for different ipsilateral veins and inferior vena cava.

Results

All 27 patients were imaged with both techniques at an average gestational age of 29 weeks (range 23-39). Three cases (11.5%) of DVT in the pelvic veins were missed on ultrasound but detected by MRI. The upper limit of the DVT was always depicted at a higher (20 cases, 65.4%) or the same level (seven cases, 34.6%) on MRI than on ultrasound. Agreement expressed as κ was 0.33 (95% CI 0.27-0.40) demonstrating only fair agreement. In one woman the thrombus had propagated into the inferior vena cava, shown only on MRI.

Conclusion

Our study suggests that in pregnant women there is only fair agreement between ultrasound and MRI for determination of extent of DVT into pelvic veins, with MRI showing consistently more detailed depiction of extension. Our results indicate that MRI has an important role as a complementary technique in the diagnosis of DVT during pregnancy.     

椎管内硬膜外血管脂肪瘤的MRI诊断

目的 探讨椎管内硬膜外血管脂肪瘤的MRI表现特征.旨存进一步提高对该病的认识. 方法 收集中山大学附属第三医院自2005年至2008年收治的2例经手术病理证实的该病瘤患者资料,回顾性分析其MRI表现特征. 结果本组2例病灶均位于胸段椎管硬膜外间隙,病灶呈梭形,长轴与脊柱纵轴平行,其MRI信号由脂肪和血管信号构成.一例向椎间孔外生长.呈"亚铃状",侵犯邻近膈肌、椎体、椎弓根及棘突;另一例病灶内见迂曲扩张的流空血管影.结论 椎管内硬膜外血管脂肪瘤具有特征性的MRI表现.MRI是诊断此病的最佳方法 .