特发性炎性肌病肌炎特异性抗体谱的临床意义

目的研究特发性炎性肌病(IIM)患者血清肌炎特异性抗体(MSAs)水平与其临床特征、实验室指标及预后的相关性。方法收集178例IIM患者的临床资料,免疫印迹法检测患者血清中12种MSAs的水平,并分析其与患者临床特征、实验室指标和预后的相关性。进一步建立Logistic回归模型,分析MSAs亚型是否为患者临床症状的独立危险因素。结果12种MSAs中,抗氨基酰tRNA合成酶[ARS,包括组氨酰-tRNA合成酶(Jo-1)、PL-7、PL-12、EJ、OJ]抗体(34.8%)最常见,其次为抗黑色素瘤分化相关基因5(MDA5)抗体(32.6%)、抗转录中介因子γ(TIF1γ)抗体(11.8%)和抗信号识别颗粒(SRP)抗体(11.8%)。抗ARS抗体阳性组肺间质性病变和技工手的发生率高于阴性组(90.3%比66.4%,P<0.001;16.1%比4.3%,P=0.007);抗MDA5抗体阳性组较阴性组更易出现肺间质性病变、Gottron疹、面部红斑和关节炎(均P<0.05),肌无力和吞咽障碍发生率更低(55.2%比78.3%,P=0.001;8.6%比23.3%,P=0.018);抗TIF1γ抗体在≥65岁患者中的阳性率高于<65岁患者(25.9%比9.3%,P=0.032),该抗体阳性组肿瘤发生率比阴性组高(19.0%比3.2%,P=0.01);抗SRP抗体阳性患者肌无力发生率、肌酸激酶(CK)值、乳酸脱氢酶(LDH)值和谷草转氨酶(AST)值高于阴性组(均P<0.05)。抗Mi-2β抗体阳性组肺间质病变发生率低于阴性组(40.0%比77.9%,P=0.003)。Logistics回归分析显示,抗MDA5抗体和抗Jo-1抗体是肺间质性病变(ILD)发生的独立危险因素,抗TIF1γ抗体是吞咽障碍发生的独立危险因素。生存分析结果表明,抗MDA5抗体阳性患者比阴性患者生存时间更短(5.0月比14.0月,P=0.001)。结论抗ARS抗体是最常见的MSAs亚型,不同的MSAs亚型与特定的临床特征有关。检测MSAs水平有利于对不同患者进行分型,指导治疗并判断预后。     

实验性血管性痴呆的模型制作问题

在亚洲及许多发展中国家血管性痴呆的发病率可能高于阿尔茨海默病，是最常见的老年期呆之一。但目前尚无用血管性痴呆基础研究的标准动物模型。本文综述了近年有关血管性痴呆的动物模型的问题。     

多发性肌炎的治疗进展

多发性肌炎是一种病因和发病机理不明的炎性肌病，目前认为与自身免疫有关。其治疗方法众说不一，除传统的皮质类固醇激素和其它免疫抑制剂外，试用血浆置换、大剂量静脉注射免疫球蛋白和放射疗法等，亦取得一定疗效。本文综述多发性肌炎近年来的治疗进展。     

肿瘤相关肌炎发病机制研究进展

从不同角度对肿瘤相关肌炎的发病机制的研究进展进行概述。通过查阅文献从临床特点、早期筛查、发病机制与基因表达、疾病的生物标志物、生存率及预后等角度进行描述。特发性炎性肌病患者存在肿瘤相关基因位点,可表达肿瘤相关自身抗体[如抗转录中介因子（TIF）1-γ抗体],某些细胞因子在体内高度表达诱发肌炎患者并发肿瘤,对TIF-1γ...     

多发性肌炎发病机制中的血管因素研究

目的　研究多发性肌炎中细胞间黏附分子 1(ICAM 1)、IL 1α在微血管的表达情况,探讨血管因素在多发性肌炎中的作用。方法　收集 10例多发性肌炎患者和 6例非炎性肌病,采用图像分析法计算CD34、ICAM 1、IL 1α阳性微血管面积与面密度,进行定量分析和比较。结果　多发性肌炎患者肌组织中微血管面密度较非炎性肌病组明显减少 (P=0 009 );微血管内皮ICAM 1、IL 1α的表达强度较对照组增强。结论　多发性肌炎患者肌组织微循环功能受损,提示血管因素可能参与了多发性肌炎的发病。     

皮肌炎/多发性肌炎患者糖皮质激素受体的研究

目的探讨皮肌炎，多发性肌炎患者外周血单个核细胞糖皮质激素受体（GR）的变化意义及其机制。方法采用放射配基结合位点分析法与反转录聚合酶链反应（RT—PCR）方法检测36例皮肌炎，多发性肌炎治疗前后及30名正常对照组外周血单个核细胞糖皮质激素受体位点数及GRmRNA表达水平，同时采用放射免疫分析法检测患者血中糖皮质激素水平。结果皮肌炎，多发性肌炎患者血中糖皮质激素水平与正常对照组相比，差异无统计学意义（P〉0．05）；而糖皮质激素受体位点数[位点，细胞]及GRmRNA表达水平，较正常对照组降低，差异有统计学意义（P〈0．01）；治疗前糖皮质激素受体位点数[位点，细胞]及GRmRNA表达水平高者，治疗效果好，而治疗前糖皮质激素受体位点数[位点，细胞]及GRmRNA表达水平低者治疗效果差。结论皮肌炎，多发性肌炎患者糖皮质激素受体位点数及GRmRNA表达水平降低，糖皮质激素受体的变化不仅与皮肌炎，多发性肌炎的发病有关，还与治疗反应有关。皮肌炎，多发性肌炎患者糖皮质激素受体位点目的减少可能与GRmRNA表达水平降低有关。     

青霉胺致多发性肌炎2例

例１：女性,４２岁。因患类风湿关节炎１０年,四肢无力,吞咽困难、呛咳２月于１９９７年６月２７日入院。患者１０年前诊断为类风湿关节炎,外院予正清风痛宁、雷公藤（３０ｍｇ／ｄ）治疗,效不佳。１９９５年２月开始改服青霉胺０２５ｇ／ｄ,两周后加至０５ｇ／...     

多发性肌炎伴心肌炎、冠脉炎一例

多发性肌炎是一种少见、难治、预后较差的结缔组织疾病。其病程短 ,病死率高 ,常累及呼吸、消化及心脏[1] 。临床上因伴有血清酶学的显著增高常误诊为急性心肌梗死[2 ] 或因全身症状严重、掩盖心脏局部症状而漏诊心脏病变。本病例在临床上排除了急性心肌梗死 ,经病理检查诊断为多发性肌炎 ,治疗无效死亡 ,后经心脏尸检发现 ,除有明显的心肌间质性炎症外 ,冠状动脉内有血栓形成。现报道如下。患者男性 ,5 2岁。因乏力、阵发性手足抽搐半年 ,加重伴反应迟钝、吞咽困难、肌肉疼痛 1个月 ,于 1998年 6月 2 9日入院。入院半年前无明显诱因出现乏…     

特发性炎性肌病分型诊断标准的比较分析

目的 评价Bohan/Peter标准(B/P标准)与欧洲神经肌肉疾病中心(ENMC)标准对特发性炎性肌病分型诊断皮肌炎和多发性肌炎的准确性.方法 回顾性收集86例初诊为特发性炎性肌病患者的临床、实验室及骨骼肌病理资料,分别用B/P标准与ENMC标准进行分型诊断,比较两个标准诊断皮肌炎和多发性肌炎的异同性.数据分析采用SPSS 13.0软件系统进行非参数检验(Mann Whitney U检验)和一致性检验(Kappa分析)方法.结果 B/P标准诊断皮肌炎37例,多发性肌炎49例;ENMC标准诊断皮肌炎46例,多发性肌炎仅14例,其余为嗜酸细胞性肌炎1例、疑诊散发性包涵体肌炎9例,未能分型者5例,肢带型肌营养不良2B型11例.Kappa分析检验两个标准诊断皮肌炎一致性较好(κ=0.79),诊断多发性肌炎一致性差(κ=0.26).结论 B/P标准对多发性肌炎存在过度诊断、误诊风险.ENMC标准纳入免疫病理,增加了临床与病理诊断的排除标准,其分型诊断准确性优于B/P标准.     

Pathogenesis of myositis: Lessons learned from animal studies

Recent studies have continued to clarify the pathogenic mechanisms responsible for muscle damage and weakness in inflammatory myopathies. Traditionally, adaptive immune mechanisms such as cell mediated (cytotoxic) and humoral (autoantibodies and complement) components have been implicated in the pathogenesis of polymyositis/inclusion body myositis and dermatomyositis, respectively. However, recent studies have shown a significant overlap of immune components in these disorders. Likewise, studies have provided evidence not only for adaptive immune pathogenic mechanisms but also for innate immune, such as the TLR-NF-κB signaling, and non-immune mechanisms, such as endoplasmic reticulum stress response, autophagy, metabolic deficits in ATP generating pathways and hypoxia. These recent studies indicate that the muscle fiber damage and weakness in myositis may not be solely mediated by an adaptive immune attack (e.g., autoreactive CTLs or autoantibodies) but also mediated through innate immune and metabolic mechanisms. In this review, we have briefly outlined the current developments in immune (adaptive, innate) and non-immune components of disease pathogenesis in inflammatory myopathies.     

A case of periodic-fever-syndrome-like disorder with lipodystrophy,myositis, and autoimmune abnormalities

A 24-year-old Japanese woman had been suffering from a periodic fever since 10 months of age. She developed deformities in her fingers, with severe atrophy of subcutaneous adipose tissue, myositis, and frostbitten hands. She showed elevated C-reactive protein, creatine kinase, and γ-globulin. She was also positive for antinuclear, anti-DNA, anti-SS-B, and anti-U1RNP antibodies. Her myositis was similar to amyopathic dermatomyositis rather than juvenile dermatomyositis. Although consanguineous marriage of her parents and early onset of disease suggested her disease as a hereditary disorder with periodic fever, her clinical feature and laboratory tests were unlike any known periodic fever syndromes. Her disease was regarded as a unique type of periodic-fever-syndrome-like disorder with autoimmune abnormalities.     

Myalgia in myositis and myopathies

Myalgia is a common symptom of various neuromuscular disorders: myalgia occurs in metabolic muscle diseases, inflammatory muscle diseases, dystrophic myopathies and myotonic muscle disorders. Myalgia leads to a significantly reduced quality of life. Other muscular symptoms that are present along with myalgia often provide the clue towards a diagnosis and include weakness, cramps and myotonia as well as the type of pain. In addition, extramuscular symptoms like an erythema in dermatomyositis can lead to the correct diagnosis. Basic diagnostic workup includes a detailed medical history, full neurologic assessment, laboratory tests, EMG and nerve conduction studies. Muscle imaging, genetic testing and muscle biopsy may be required to make a diagnosis. Whenever possible, treatment should aim to improve or correct the underlying cause for myalgia such as inflammation or hypothyroidism. Symptomatic therapy includes different avenues: Myotonia can be treated with mexiletine. Carbamazepine or phenytoin can be used in myotonic syndromes, particularly with muscle cramps. Pregabalin, gabapentin, or amitriptyline can be tried in conditions with myalgic pain. This review summarizes the symptoms, diagnostic strategies, and therapeutic approach in neuromuscular disorders that present with myalgia.     

Paraneoplastic myalgias and myositis

There are several key observations on paraneoplastic myopathies: The risk of cancer associated with DM is very high, whereas risk of cancer associated with PM is mildly increased Most cancers develop within one year of the onset of myositis, although the risk remains high up to 5 years after diagnosis. The most common cancers associated with DM are adenocarcinoma, including lung, ovary, cervical, stomach, pancreas, colorectal and lymphoma, whereas PM is associated with a high risk of lymphoma. The clinical course of myopathy is closely linked with the course of cancer. Certain clinical features are associated with CAM including severe treatment resistant skin manifestations, severe muscle weakness, respiratory muscle weakness, and dysphagia, while some clinical features are protective such as arthritis, Raynaud, and ILD. Screening should be based on age, gender, ethnicity, and the geographic area of the patient; however, certain high-risk patients may require more extensive screening including tumor markers and thoracoabdominal–pelvic CT scans. Certain autoantibodies including anti-p155 and the absence of more common autoantibodies are associated with a higher risk of CAM, while the presence of antisynthetase autoantibodies lowers the risk for CAM. Although the pathogenesis of CAM is unclear, a plausible hypothesis is that immune responses generated against antigens commonly targeted in myositis are related to antitumor responses in affected individuals.     

一种实验性大鼠慢性阻塞性肺疾病模型的建立

目的 :建立实验性大鼠慢性阻塞性肺疾病 (COPD)模型。方法 :采用香烟染毒和细菌内毒素 (LPS)气管注入的复合方法 ,模仿人类COPD发病的慢性过程 ,制造大鼠慢性阻塞性肺疾病模型。结果 :COPD模型肺组织平均肺泡面积较健康对照组明显增大 ,OD值明显减少 (P <0 0 1)。COPD模型组吸气阻力较健康对照组显著升高 ,肺顺应性 (CLd)及第 0 3s用力呼气量 (FEV0 3)与用力肺活量的比值(FEV0 3 FVC % )较健康对照组显著降低 ,表明有通气阻塞现象 (P <0 0 1)。结论 :本组大鼠COPD模型病理形态学改变和肺功能均与人类慢性阻塞性肺疾病的改变基本一致     

Pitfalls in the diagnosis of myositis

The idiopathic inflammatory myopathies are a group of heterogeneous autoimmune connective tissue diseases. Despite increase in the understanding of these conditions, securing a timely diagnosis and accurate subtype classification remains difficult in some cases. This has important implications for patients, where delayed or inappropriate treatments can have a negative effect on outcomes.Several conditions can mimic myositis, including metabolic myopathies, genetic myopathies and neurological disease. In addition, the heterogeneity within the idiopathic inflammatory myopathy spectrum can also create diagnostic confusion, referred to here as ‘myositis chameleons’. This includes inclusion body myositis, immune-mediated necrotizing myopathy, hypomyopathic variants of anti-synthetase syndrome and overlap disease.We highlight the importance of a thorough diagnostic workup, refer to updated classification criteria and emphasize the importance of myositis autoantibody testing. Where diagnostic doubt exists, the involvement of a specialist centre and a multidisciplinary team is vital.     

Localized nodular myositis. A paraneoplastic phenomenon

Summary Localized nodular myositis was recognized in an elderly man six months prior to the diagnosis of Hodgkin's disease. Meticulous search of the muscle specimen failed to disclose tumorous involvement. The possible paraneoplastic nature of localized nodular myositis in this patient is discussed.     

降低大鼠心肌梗死模型死亡率的方法

目的 探讨降低大鼠心肌梗死模型死亡率的影响因素.方法 结扎180只Wistar雄性大鼠左冠状动脉前降支制备心肌梗死模型,分析麻醉药品、气管插管方法、结扎部位的高低及术后护理等因素对大鼠死亡率的影响,并取心脏组织行HE染色、氯化四唑（tetrazolium chloride,TTC）染色确认模型成功建立.结果 水合氯醛麻醉死亡率15%,戊巴比妥钠麻醉死亡率35%,差异有统计学意义（P＜0.05）;经口气管插管大鼠死亡率10%,经气管切开插管死亡率29.4%,差异有统计学意义（P＜0.05）;有术后护理大鼠死亡率5.9%,无术后护理大鼠死亡率22.2%,差异有统计学意义（P＜0.01）.而结扎部位的不同对大鼠手术死亡率及手术成功率亦有明显的影响.结论 本研究显示麻醉药品、气管插管方法、结扎部位的高低及术后护理等因素均对梗死模型的死亡率有影响,选择合适的麻醉药品和剂量以及气管插管方法,正确的结扎部位及适当的术后护理等可明显降低制作大鼠心肌梗死模型死亡率.     

Facioscapulohumeral dystrophy with myositis associated with rheumatoid arthritis

Summary We report a case of facioscapulohumeral dystrophy with myositis, rheumatoid arthritis and serologic findings suggestive of autoimmune disease. To our present knowledge this is the first time this association has been observed.     

Focal myositis: A pseudotumoral form of polymyositis

Summary The authors report a case of pseudotumoral focal myositis found in a 55 year-old man, in the anteroexternal area of the right leg. The diagnosis was based on the pathological findings, the absence of any further clinical signs or laboratory findings, and no further development over time. They compare their patient's characteristics with cases found in the literatur (24 cases during the past 20 years). Diagnostic certainty confirms the benign character of this pathology, which contrasts with the gravity of the classical form of polymyositis.