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。     

间质性肌炎的临床和病理特征

目的　总结间质性肌炎 ( interstitial myositis,IM)临床特点及病理特征 ,探讨 IM的诊断、治疗效果和预后。方法　综合分析作者医院 2 3例 IM的临床资料 ,并与 93例多发性肌炎、3 4例皮肌炎进行比较。结果　 IM临床表现以肌无力、肌肉疼痛、血清酶谱增高、肌电图及病理学异常为特征 ,治疗以激素为主。结论　病理学检查对 IM诊断及疗效判断有重要价值。     

坏死性肌病9例临床与病理分析

目的探讨坏死性肌病的临床和病理特点及其对药物的治疗反应.方法采用回顾性研究方法.结果患者的临床表现同多发性肌炎(PM),肌肉活检显示肌纤维变性、坏死,部分有吞噬现象,但无炎性细胞浸润;患者对激素联合免疫抑制剂治疗有效;年龄较大、病程较长、用药较晚者治疗效果差.结论坏死性肌病可能为PM的特殊类型,患者对激素合并使用免疫抑制剂有效.     

多发性肌炎/皮肌炎临床和病理研究(附305例分析)

目的:研究多发性肌炎/皮肌炎(PM/DM)的临床、病理特征。方法:回顾性总结305例PM/DM患者的临床资料,根据Bohan标准分为5型,研究其临床表现、血清肌酶学、肌电图、肌肉病理的特点。结果:本病临床上主要有肌无力、肌痛或肌捏痛,CK等血清肌酶增高,肌电图呈肌原性损害。各型肌肉病理均显示免疫炎性改变,以单纯多发性肌炎组织损害程度较重,主要表现为肌纤维散在萎缩、肌肉膜炎;皮肌炎组多为肌束周萎缩、血管炎性病变;肌炎合并恶性肿瘤(CAM)、儿童型肌炎(JPM/DM)、肌炎合并其它结缔组织疾病(CTM)三组血管炎性改变亦较明显。结论:各型肌炎的临床和病理有所不同,发病的免疫病理机制亦有所区别。     

多发性肌炎15例临床与肌肉病理

多发性肌炎１５例临床与肌肉病理孙东华，高凤琴，李维起多发性肌炎和皮肌炎是累及骨骼肌的非特异性炎性疾病。其诊断标准是：（１）近端肌肉无力伴肌痛或萎缩。（２）肌纤维灶性坏死、再生和单核细胞浸润。（３）血清酶谱ＣＰＫ升高。（４）肌电图有多灶性肌原性改变。病...     

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。     

脂质沉积性肌病2例临床与病理报告

例１,女性,２８岁。病人于２年前无诱因的开始出现四肢近端和颈部肌肉无力,有时伴有相应部位的肌肉压痛。曾诊断为“多发性肌炎”服用强地松后症状好转,但症状反复出现,且有加重的趋势。入院查体：神志清晰、言语流利。耸肩、转颈无力,四肢近端肌力Ⅲ级,远端肌力Ⅳ级,肌张力低,双上臂肌肉压痛,无肌肉萎缩。四肢腱反射减弱,病理反射未引出。实验室检查：乳酸脱氢酶（ＬＤＨ）６９６Ｕ／Ｌ（正常值６０－１４０Ｕ／Ｌ）,血清肌酸磷酸激酶（ＣＰＫ）７６０Ｕ／Ｌ（正常值１５－１３０Ｕ／Ｌ）,肌酸磷酸激酶同功酶（ＣＫ－ＭＢ）５…     

误诊为多发性肌炎的线粒体肌病1例临床病理报告

线粒体肌病是一组包括神经系统和肌肉为主的多系统线粒体结构、功能和生化代谢失常的疾病,文献已有较多报道。其临床表现复杂,伴有多种综合征,其中以眼肌麻痹,伴骨骼肌无力症状为最多见,称为线粒体肌病。多表现为近端肌无力和肌疲劳现象,且常常呈周期性发作,易误诊为“多发性肌     

3例中央核肌病的临床和病理特点

目的 报道3例中央核肌病的临床和病理特点，讨论其分类和可能的发病机制。方法 3例患儿均在生后发病，表现为运动发育延迟和骨骼畸形，肌无力随年龄的增加而逐渐好转，肌酶正常或轻度升高，肌电图呈肌源性损害。对3例患者进行肌肉活检，肌肉活检标本做组织学和酶组织化学染色。结果 肌肉病理发现在许多肌纤维的中心出现单个的肌核(15％一31％)，主要累及Ⅰ型肌纤维，伴随Ⅰ型纤维呈病理性占优势和Ⅰ型肌纤维发育不良为主的肌型比例失调(Ⅰ型肌纤维直径显著小于Ⅱ型肌纤维)。结论 肌肉活检证实这3例患者为中央核肌病，可能属于预后良好的常染色体隐性遗传型。中央核肌病可以出现腓肠肌肥大、肌型比例失调以及Ⅰ型肌纤维病理性占优势是此病常见的病理改变。     

特发性炎症性肌病所致精神障碍1例

1病例患者男,42岁。于2年前无明显原因四肢肌无力,下蹲、起立、举臂,平卧、抬头均困难,肌肉压痛。心电图示窦性心律不齐,肌酶升高。肌电图示肌原性损害。2周后突然害怕,怕别人害他,发脾气,大喊大叫,凭空能够听见有人骂他。傻笑,否认有病。既往史,个人史,家庭史无特殊。病前性格     

The role of interleukin-17 in immune-mediated inflammatory myopathies and possible therapeutic implications

The idiopathic inflammatory myopathies are a heterogeneous group of autoimmune muscle disorders with distinct clinical and pathological features and underlying immunopathogenic mechanisms. Traditionally, CD4⁺ Th1 cells or CD8⁺ cytotoxic effector T cells and type I/II interferons have been primarily implicated in the pathogenesis of the inflammatory myopathies. The presence of IL-17A producing cells in the inflamed muscle tissue of myositis patients and the results of in vitro studies suggest that IL-17A and the Th17 pathway may also have a key role in these diseases. The contribution of IL-17A to other chronic inflammatory and autoimmune diseases has been well established and clinical trials of IL-17A inhibitors are now at an advanced stage. However the precise role of IL-17A in the various forms of myositis and the potential for therapeutic targeting is currently unknown and warrants further investigation.     

Seasonal occurrence of relapses in inflammatory myopathies: a preliminary study

The seasonal occurrence of relapses was analysed retrospectively in a group of 53 patients with treated dermatomyositis (DM) or polymyositis (PM). In DM, the incidence of both myositic and cutaneous relapses was highest in summer whereas in the PM group relapses was more evenly distributed throughout the seasons but lowest in summer. The present findings suggest that environmental factors such as intercurrent infections and light exposure may be involved in reactivating the disease process and causing relapses in DM but less so in PM. Further prospective studies are needed to assess the role of environmental factors in the initiation and reactivation of the inflammatory myopathies. Received: 12 May 2001, Received in revised form: 23 August 2001, Accepted: 27 August 2001     

胶原血管病伴肌炎69例临床和病理研究

目的:研究胶原血管病伴肌炎(CTM)的临床、病理特征。方法:回顾性总结69例CTM患者的临床资料,研究其临床表现、血清免疫学、肌酶学、肌电图、肌肉病理的特点。结果:本病临床上主要有肌无力、肌痛、关节痛、发热、肌萎缩、雷诺征等;血清免疫学可见免疫球蛋白增高和补体降低,部分患者ANA、ENA阳性;CK等血清肌酶增高不及单纯性多发性肌炎(PM)明显;肌电图呈肌原性损害,也可有神经原性损害。肌肉病理显示免疫炎性改变,表现为区域性肌纤维变性坏死,肌间质血管狭窄、闭塞、炎症细胞浸润,血管炎和肌束周萎缩远较PM常见(P<0.01)。结论:CTM的临床和肌肉病理与PM有所不同,广泛的血管炎性病变是CTM特征性的病理改变。     

Inflammatory myopathies and systemic disorders: a review of immunopathogenetic mechanisms and clinical features

The inflammatory myopathies are a heterogeneous group of muscle diseases characterized by muscle degeneration mediated by inflammatory processes. They may be idiopathic, as in polymyositis, dermatomyositis and inclusion body myositis, or associated with systemic disorders such as malignancies, overlap syndromes, and retroviral infection. The pathogenesis of each disease is discussed together with more recent molecular and cellular immunology findings. Salient diagnostic, clinical and pharmacological features are also reviewed. Received: 28 September 1996 Accepted: 18 October 1996     

Pleomorphic mitochondrial and different filamentous inclusions in inflammatory myopathies associated with mtDNA deletions

Mitochondrial changes are frequently observed in muscle fibers of patients with inclusion body myositis (IBM) and polymyositis (PM), suggesting that mitochondrial function may be especially impaired in these forms of inflammatory myopathies. Intranuclear and cytoplasmic tubulofilamentous inclusions are characteristic, although not totally specific for IBM. In the present cases, the inclusions were strikingly pleomorphic when chloroquine had been given for long periods. The nuclear inclusions were always tubular, whereas the cytoplasmic filaments had either a tubular, a helical, or a cross-striated structure with different diameters and arrangements in association with myelin-like figures, and vacuoles. Abnormal mitochondria containing paracrystalline, globoid, and other inclusions, noted in IBM, were occasionally also seen in PM or vasculitis. By contrast, in the latter, no intranuclear or cytoplasmic tubulofilamentous inclusions were apparent in muscle fibers. This study reports for the first time the presence of membrane-bound crystalloid inclusions in a muscle fiber with numerous abnormal mitochondria; similar structures have thus far only been observed in macrophages. The identity and function of these inclusions remains unknown. Using PCR analysis we detected different mtDNA deletions not only in IBM, but also in PM and vasculitis, indicating at least some degree of association between the structural mitochondrial abnormalities and the mtDNA mutations. There was no topographical correlation between the presence of tubular or helical filaments and the mitochondrial abnormalities. As already noted by others, the mitochondrial changes in IBM were more frequent than expected in this age group. It is suggested that the presence of the mtDNA deletions in IBM and PM are not primary, but rather the result of the underlying, presumably immunological disorder causing nuclear and secondary or simultaneous mitochondrial changes. Received: 12 May 1997 / Revised, accepted: 17 February 1998     

Expression and distribution of the nitric oxide synthases in idiopathic inflammatory myopathies

Different immune effector mechanisms have been characterised in the idiopathic inflammatory myopathies (IIM): in polymyositis (PM) and sporadic inclusion body myositis (sIBM), T-cell-mediated cytotoxicity targets nonnecrotic muscle fibres, whereas in dermatomyositis (DM) the complement-mediated immune response is directed against the microvasculature. As nitric oxide (NO) has an important function in cell signalling and in the cytotoxicity displayed by activated macrophages, it is potentially involved in the immunopathogenesis of IIM. Using immunohistochemical, in situ hybridisation and Western blotting techniques, we visualised the three isoforms of NO synthase (NOS) in muscle tissues from normal controls and from patients diagnosed with IIM. The levels of both constitutive isoforms of NOS (endothelial, i.e., eNOS, and neuronal, i.e., nNOS) were unchanged in IIM as compared with normal muscle. Both protein and mRNA of the inducible form (iNOS) were detected in half of the control biopsies. Constant and increased iNOS protein expression was found in endomysial infiltrates of PM and sIBM, whereas perimysial inflammatory cells in DM were largely negative. We developed a quantitative Western blotting protocol which confirmed the constitutive nature of nNOS and eNOS and the significant induction of iNOS in PM. Our results appoint iNOS with a dual function: a limited and transient role in normal muscle physiology and an active cytotoxic role in PM and sIBM.     

Demographic and clinical determinants of the quality of life in adults with inherited and acquired myopathies

Background and purpose

Measuring health-related quality of life (QOL) is vital for understanding the disease impact, but the complex relationship between clinical parameters and QOL remains unclear. The objective was to determine the demographic and clinical factors that influence the QOL in adults with inherited and acquired myopathies.

Methods

The study was of cross-sectional design. Detailed demographic and clinical details were collected. Patients answered Neuro-QOL and Patient-Reported Outcomes Measurement Information System short-form questionnaires.

Results

Data was collected from 100 consecutive in-person patient visits. Mean age of the cohort was 49.5 ± 20.1 (18–85) years, and the majority were male (53, 53%). Bivariate analysis between the various demographic and clinical features with the QOL scales revealed single simple question (SSQ), handgrip strength, Medical Research Council (MRC) sum score, female gender, and age to be nonuniformly associated with the QOL scales. There was no difference between inherited and acquired myopathies for any of the QOL scores, except for the poorer lower limb function domain in inherited myopathies (36.7 ± 7.3 vs. 40.9 ± 11.2, p = 0.049). Linear regression models revealed lower SSQ, lower handgrip strength, and lower MRC sum score to independently predict poor QOL.

Conclusions

Handgrip strength and SSQ serve as novel predictors of QOL in myopathies. Handgrip strength has a significant impact on physical, mental, and social domains and deserves special attention with respect to rehabilitation. SSQ correlates well with QOL and can be employed as a quick and global assessment of a patient's well-being. Differences in QOL scores between patients with inherited and acquired myopathies were minimal.