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。     

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。     

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.     

多发性肌炎与皮肌炎的实验室检查分析

分析了４３例多发性肌炎和皮肌炎的肌酶谱、肌电图和肌肉病理检查对本病的特异性。结果肌酶中最敏感的ＣＰＫ升高者占７５．６％。肌肉病理典型改变者占７１．４％，肌电图表现为肌原性损害者占４６．２％。结合文献探讨了三项实验室检查的局限性和多发性肌炎与皮肌炎的区别。     

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。     

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.     

影响多发性肌炎和皮肌炎疗效的因素分析和对策

目的探讨影响多发性肌炎和皮肌炎疗效的因素和对策。方法通过对66例多发性肌炎和皮肌炎的临床资料和治疗方法进行分析。结果延误诊断、糖皮质激素用量不足和疗程不够、不及时加用免疫抑制剂、伴发肺间质病变或心肺功能差及恶性肿瘤是影响其疗效的因素。结论及早诊断,早期足量,尤其早期激素冲击并继之以长时间适当剂量糖皮质激素,及时加用其他免疫抑制剂、尽早发现肺间质疾病与排除恶性肿瘤,可提高疗效,改善预后。     

60例多发性肌炎和皮肌炎治疗体会

多发性肌炎(PM)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,如同时累及皮肤则称为皮肌炎(DM).现已明确本病是具有遗传易感性的个体被各种环境因素触发后产生的免疫介导过程.皮质激素及免疫抑制剂常作为本病的首选药物,但由于用药方法不同对其远期疗效影响颇大,至今尚无公认或普遍适用的治疗方案.本文总结自1990年以来收治的60例PM和DM,着重对首发和复发患者的治疗进行讨论.     

磁共振成像在多发性肌炎、皮肌炎诊断中的应用

目的 研究多发性肌炎(PM)、皮肌炎(DM)的磁共振成像(MRI)表现，评价MRI在PM及DM诊断中应用价值。方法 对5例PM及4例DM患者行MRI检查，选择骨盆、双侧大腿及小腿肌肉，采用自旋回波序列(SE)、快速自旋回波序列(FSE)及短时反转恢复序列(STIR)进行扫描。结果 5例PM及4例DM患者的受累肌群均表现为斑片状等T1长T2异常信号，2例PM患者尚可见小斑片状短T1长T2异常信号影；6例患者肌筋膜增厚，呈长T2线样高信号改变；4例DM患者的表皮及皮下结缔组织尚可见条带状及网格状长T1长T2异常信号影。受累肌群主要表现为炎症水肿样改变，晚期受累肌肉可见少量脂肪替代改变；病变双侧不对称，以内收肌受累最重，股中间肌受累最轻。结论 PM与DM患者的MRI表现具有一定的特征性，MRI能为PM及DM的定位诊断、疗效判断及病情随访提供客观资料，并能为临床选取准确的活检部位提供帮助。     

Quantitative electromyography in polymyositis and dermatomyositis

Twelve patients with polymyositis or dermatomyositis were examined repeatedly during the course of the illness by muscle strength tests, serum enzyme determinations, frequency analysis of the electromyographic signal, and estimations of the spontaneous activity. All methods can be used to follow the course of the disease. Increasing muscle strength was correlated to decreasing serum enzyme values, decreasing spontaneous activity, and decreasing proportion of high-frequency components in the EMG signal, except during the initial period of treatment, when a temporary increase in high-frequency components was recorded.     

Muscle biopsy findings predictive of malignancy in rare infiltrative dermatomyositis

Objective

The characteristic pathological muscular findings of polymyositis (PM) and dermatomyositis (DM) have been shown to reflect their different pathogeneses. Here, we characterized the muscle biopsy findings of PM and DM patients with or without malignancy.

Methods

We evaluated the muscle biopsy findings of 215 consecutive PM and DM patients admitted to our hospital between 1970 and 2009. Pathology of the lesion biopsy sections was classified into 3 types: endomysial infiltration-type, perivascular infiltration-type, and rare-infiltrative-type.

Results

There was no difference between the muscle pathology of PM patients with and without malignancy. However, the incidence of rare-infiltrative type muscle pathology in DM patients with malignancy was significantly higher than in those without such tumors (p = 0.0345).

Conclusion

The incidence of rare-infiltrative type muscle pathology may be a predictive marker of DM with malignancy.     

Polymyositis presenting with cardiac manifestations: Report of two cases and review of the literature

We report two patients with severe cardiac manifestations at the time of the initial presentation of polymyositis. Both cases are unusual in that they presented with predominant cardiac disturbances, associated with muscle weakness. One patient had a typical clinical syndrome of congestive heart failure, and the second mimicked an acute myocardial infarction in which coronary angiography was normal. From our cases, we can emphasize that aside from characteristic symmetrical proximal muscle weakness, the clinical features of polymyositis may also include cardiac complications.     

慢性多发性肌炎临床及病理分析

目的探讨慢性多发性肌炎的发病机制、临床和病理特征。方法回顾性分析95例慢性多发性肌炎患者临床表现、肌酶学和肌电图检查结果,总结肌肉病理学特征。结果慢性多发性肌炎以四肢近端肌无力、肌萎缩为主要表现,血清酶谱轻-中度增高,肌电图以肌源性损害为主,病理改变为灶性坏死、炎性细胞浸润与再生肌纤维共存。结论临床特点结合病理学检查有助于慢性多发性肌炎的诊断,多数患者激素治疗有效。     

伴有眼外肌麻痹的多发性肌炎

目的探讨多发性肌炎眼外肌受累的特点。方法收集1例以眼外肌麻痹为突出表现的多发性肌炎叠加综合征患者临床病理资料并结合文献总结在炎性肌病中眼外肌受累的特点。结果（1）实验室检查：肌酸激酶6498U/L,抗核抗体（1：320）阳性（均质型）,肌电图提示肌源性受损,重频刺激试验阴性；（2）肌肉活检：可见明显的肌纤维变性坏死、吞噬现象及局灶性淋巴细胞浸润,MHC-I免疫组化染色可见广泛肌纤维图MHC-I表达阳性以及炎性细胞浸润MHC-I表达阳性的正常肌纤维。结论眼外肌麻痹是炎性肌病的少见表现。在眼外肌麻痹的鉴别诊断时应考虑到炎性肌病的可能性。     

Clinico-developmental aspects in 44 cases of polymyositis/dermatomyositis

A retrospective study, paying particular attention to the clinical and evolutive aspects of the disease, was performed on 44 subjects affected by polymyositis/dermatomyositis (PM/DM) and hospitalized at the Institute of Medical Clinics of the 1st School of Medicine of Naples University. On the basis of the different clinical pictures, the cases were classified into the following groups: primary PM (4); primary DM (19); DM/PM associated with malignancy (6); childhood DM/PM (3); PM/DM associated with connective tissue disorders (12). Diagnosis was established in terms of the following criteria: a) symmetrical and mostly proximal bilateral muscle weakness (100%); b) elevation of serum enzymes (86.3%); c) electromyographic findings of myopathy sometimes with fibrillation potentials, increased insertional irritability and pseudo-myotonic discharges (93.1%); d) muscle biopsy changes compatible with a clinical form of polymyositis (83.3% out of 30 cases); e) dermatological manifestations including particularly pink or lilac edema-erythema over the periorbital areas, wine-red maculae, Gottron's sign, "poikiloderma vasculare atrophicans", telangiectasias and skin vasculitis (86.3%). An involvement of the extraneural apparatus and organs was present in 40 patients; the most damaged was the osteoarticular apparatus, followed by esophagus, lung, heart and kidney; such pathology was rarely present in the childhood form. A follow-up of the disease has been performed in 36 cases and the therapy consisted fundamentally of high dose corticosteroids (mostly prednisone), associated, in a minority of cases, with methotrexate. A clinical improvement was observed in most cases and a remission of the disease in part of the latter. However, a worsening of the illness was noticed only in the patients suffering from PM/DM associated with malignancy, and mortality rate was 11.1% in all.