神经肌炎——一种新型的炎性肌病?

多发性肌炎和皮肌炎(polymymitis/dennatomyositis,PM/DM)均属于特发性炎性肌病(idiopathic inflammatory myopathy),该病主要以近端肌无力及骨骼肌非化脓性炎症为特征,如肌炎同时伴有多种形态的皮疹者,称为皮肌炎.     

表现似运动神经元病的慢性多发性肌炎一例

表现似运动神经元病的慢性多发性肌炎一例黎莉，李大年，吴金玲，张松现将我室经病理证实貌似运动神经元病的一例慢性多发性肌炎报告如下。患者，男，３０岁，四肢进行性无力、肌萎缩１０年。１０年前无明显诱因出现左手无名指和小指无力，逐渐累及前臂和上臂，伴明显肌肉...     

Th17细胞与Treg细胞在肌肉疾病免疫机制中作用的研究进展

在原有的Th17细胞发挥促炎性作用及Treg细胞发挥免疫抑制作用的理论基础上,近年来,探寻Th17细胞和Treg细胞在多种免疫性疾病的发生、发展中所起到的作用已成为临床研究的一大热点。多项针对两种细胞的致病机制的研究表明,Th17细胞和(或)Treg细胞表达量的失衡可用来解释部分免疫性肌肉病的发病。本文从总结Th17细胞和Treg细胞的分化来源及相互作用的功能入手,对两种细胞在肌肉病免疫致病机制中的作用进行综述。     

以心肌炎为突出表现的特发性炎性肌病5例分析

目的 报道一组以心肌炎为突出表现的特发性炎性肌病(IIMs)患者的临床与病理特点。方法 回顾性分析2017年1月至2021年11月北京大学第一医院就诊的5例IIMs合并心肌炎患者的临床、血清学以及病理学资料。结果 5例患者中,男3例、女2例,发病年龄47～70岁,中位数为58岁,发病至确诊的时间为2～49个月,中位时间为21个月。3例患者慢性起病,以活动性喘憋、胸闷为突出表现,伴轻度骨骼肌力弱,血清抗线粒体抗体(AMA)阳性,心脏成像检查显示扩张性心肌病;1例患者亚急性起病,以力弱、呼吸困难为主要表现,血清抗信号识别颗粒(SRP)抗体弱阳性;1例患者急性起病,表现为晕厥、胸闷,四肢肌力正常,自身抗体检测阴性,肌电图检查显示右胫前肌呈肌源性损害。5例患者行骨骼肌活检,均呈炎性肌病病理改变。5例患者均接受免疫治疗,除1例在短暂好转后出现恶性心律失常而猝死外,其余4例症状均明显改善。结论 部分IIMs患者以心肌炎为突出表现,伴或不伴四肢力弱,AMA阳性多见,外周组织骨骼肌病理检查有助于明确诊断。     

慢性移植物抗宿主病相关性多发性肌炎一例临床和骨骼肌病理特点

目的 报道1例慢性移植物抗宿主病相关性多发性肌炎患者的临床和骨骼肌病理改变特点.方法 我院于2010年12月29日收治1例慢性移植物抗宿主病相关性多发性肌炎患者,该患者为女性,40岁,因急性粒单核细胞白血病于20个月前行异基因造血干细胞移植术.术后给予环孢素A抗排异治疗13个月,6个月前缓慢出现进行性四肢近端无力,伴有肌痛.血肌酸激酶升高(426～1948 U/L).血清抗EJ抗体强阳性.肌电图提示神经源性损害,周围神经传导速度下降.对该患者进行左肱二头肌活体组织检查,标本除进行组织学、酶组织化学染色外,还采用抗CD8、CD20、CD68和主要组织相容性复合物-Ⅰ (MHC-Ⅰ)鼠抗人单克隆抗体作为第一抗体进行免疫组织化学染色.结果 骨骼肌的主要病理改变是肌纤维直径变异加大,伴随肌纤维坏死、再生以及成小组分布的角状萎缩肌纤维.血管周围和肌内衣可见呈灶性分布的CD8+T淋巴细胞和CD6+8单核细胞浸润.MHC-Ⅰ染色显示大部分肌纤维膜异常着色.结论 慢性移植物抗宿主病相关性多发性肌炎具有慢性炎性肌肉病特点,可以伴随神经源性骨骼肌损害.     

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。     

普通变异型免疫缺陷合并肌肥大强直性炎性肌病一例

目的 报道1例出现肌肥大和肌强直的普通变异型免疫缺陷(common variable immunodeficiency,CVID)伴随炎性肌肉病患者.方法 患者男性,33岁,近10年反复出现呼吸道感染症状,出现四肢无力和肌肉强直2年,伴随胸锁乳突肌和胫前肌肥大.对患者进行实验室检查,肌电图以及肌肉超声检查,并行股四头肌活体组织检查,标本进行组织学和标记炎细胞的免疫组织化学染色,以及强直性肌营养不良1型的萎缩性肌强直蛋白激酶基因的CTG重复次数和2型锌指蛋白9基因的CCTG重复次数的基因检查.结果 肌电图检查示强直电位和肌源性损害,肌肉超声提示肌肉肥大,肺活体组织检查提示炎性改变.辅助检查示贫血、尿蛋白阳性和血免疫球蛋白IgG、IgA和IgM均降低.肌肉活体组织检查可见肌纤维坏死和再生,伴随间质增生和大量炎细胞浸润.浸润的炎细胞主要是巨噬细胞和T淋巴细胞.存在主要组织相容性复合物Ⅰ阳性肌纤维.强直性肌营养不良1型和2型的基因检查正常.结论 CVID伴随的炎性肌肉病可以出现肌强直和肌肥大表现.     

表现为多发性肌炎的脂质沉积性肌病2例报告

表现为多发性肌炎的脂质沉积性肌病２例报告赵兵国，蒲传强，朱克例１患者男性，２４岁，主因反复四肢及颈部肌无力伴吞咽困难４年人院．自１９９０年３月无明显诱因自觉双下肢无力，呈进行性加重，８个月后只能行走数１０米，不能上楼．抬头困难，咀嚼无力，吞咽困难。外...     

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     

以脊髓浸润为首发症状的慢性淋巴细胞白血病一例

1病例报告患者男,62岁。因“双下肢无力2个月余”于2011‐12‐25入院。入院前3个月无明显诱因下出现腰腹部束带感,持续3～4d后自行缓解;入院前2个月出现左下肢乏力并逐渐加重;入院前1个月左下肢已不能抬起,并出现右下肢乏力、排便困难。期间行腰椎M RI检查示轻度腰椎间盘突出,尝试针灸及牵引治疗均无好转;入院前1周行颈胸髓M RI示颈髓及胸髓内可见多发斑片状稍长 T1、稍长T2信号病灶,增强后无明显强化,予以地塞米松、甘露醇、B族维生素治疗,患者双下肢无力部分好转。为进一步诊治收入作者医院。入院体检：体温36.6℃,血压120／80 mmHg ,意识清楚,全身浅表淋巴结未触及肿大,肝脾肋下未触及,脑神经（－）,双上肢及右下肢肌力V级,左下肢肌力V －级,胸6水平以下针刺觉及振动觉减退（左侧尤为显著）,四肢腱反射（＋＋）,双侧病理征阴性。入院后血常规检查结果显示：白细胞29.6×109／L ,淋巴细胞绝对值21.9×109／L ,血红蛋白137 g／L ,血小板193×109／L。外周血流式细胞学检查结果：B细胞型CD19、CD20、CD79a均为（＋）;CD5、CD23、CD34、CD10均（－）。腰穿脑脊液检查结果：脑脊液无色透明,白细胞260×106／L ,淋巴细胞100％;葡萄糖、蛋白及氯化物均正常。脑脊液流式细胞学检查：B细胞型,CD19、CD20、HLA‐DR均（＋）;CD5、CD23、CD34、CD10均（－）。骨髓细胞学检查：淋巴细胞比例增高,占56.5％;骨髓增生明显活跃,中性粒细胞比例减低,红细胞系比例减低,巨核细胞增生活跃,血小板可见。骨髓活检检查：骨髓增生明显活跃,淋巴细胞比例增高明显。骨髓染色体检查：正常,核型46,XY。诊断：慢性淋巴细胞白血病（CLL）。2012‐01起予以FR方案化疗〔氟达拉滨40 mg（第1～3天）＋利妥昔单抗600 m g （第1天）〕,1疗程／4周,共6疗程;每次化疗同时给予鞘内注射地塞米松5 mg、甲氨蝶呤10 m g。化疗第1个疗程后患者神经系统症状体征完全消失,第3个疗程后复查脑脊液、血常规均正常。此后每半年定期随访,末次随访为2014‐03,血常规及脑脊液检查结果正常,脊髓M RI检查示髓内病灶完全消失,处于完全缓解期。     

Dermatomyositis, lobar panniculitis and inflammatory myopathy with abundant macrophages

Dermatomyositis (DM) is a rare treatable muscle disorder with a reported favorable outcome in most patients. A localized skin/muscle involvement in a DM patient raises questions of definition and causes such as lymphoma, or relapse. We describe here a young treated DM patient who presented a focal biopsy-proven destructive myositis and dermatitis restricted to the left thigh 15 months after the onset of a treated dermatomyositis. There was evidence of subcutaneous lobular panniculitis, somewhat resembling cytophagic histocytic panniculitis associated with a focal inflammatory myopathy with abundant macrophages that destroyed the sartorius muscle. Mild signs of hemophagocytosis and T-CD3 lymphocytosis were present in the bone marrow, but no monoclonal T-lymphocyte expansion was observed, as searched by autoradiography of the totality of TcR Vgamma families. The patient improved with prednisone and azathioprine. We conclude that this focal complication suggests a continuum between dermatomyositis, CHP, and IMAM, the three syndromes where T-cell activation plays an important role.     

脂质沉积性肌病(附1例报道)

目的报道1例临床上少见的脂质沉积性肌病(lipid storage myopathy,LSM)患者的临床资料,结合相关文献分析其临床特点、病理特征及治疗效果。方法对1例LSM患者的临床表现、肌电图、实验室检查、及肌肉活检等方面进行了探讨。结果患者临床表现为四肢近端肌无力和对运动不耐受;肌电图示肌源性损伤;血清肌酶都有不同程度的升高;肌肉活检显示肌纤维内有大量脂滴沉积。予低脂、高肉毒碱饮食,肾上腺皮质激素、B族维生素、辅酶Q10等治疗后,患者症状缓解。结论 LSM是一种以肌无力和运动易疲劳为主要临床表现的脂质代谢障碍性肌病,诊断需依赖症状、体征、肌酶、肉毒碱检查和肌电图、肌活检等综合评价。其中肌活检发现肌纤维内脂质沉积是目前临床上诊断此病的重要方法和指标。肾上腺皮质激素、肉毒碱、维生素、及含有丰富肉毒碱食物等综合治疗对改善临床症状有效。     

成人散发型杆状体肌病1例报道

Shy 等[1]于1963年首次报道了一类少见的具有遗传异质性的先天性肌肉疾病：杆状体肌病,主要以肌纤维内大量异常沉积的杆状小体为特征。现对解放军第八九医院收治的一例成人散发型杆状体肌病进行报道,并结合近年国内外文献共同探讨该病发病机制、临床表现、病理学特征及治疗进展。     

Human myoblasts modulate the function of antigen-presenting cells

Muscle biopsy specimens of myositis patients were analyzed for the presence of dendritic cells (DC) and macrophages (MPh) by immunohistochemistry. The interaction of DC and myoblasts (MB) was studied by coculture and effects on DC phenotype and function were assessed by flow cytometry and T-cell proliferation assays. Effects of MB-lysates on the phagocytic capacity of MPh were analyzed in bead-incorporation assays. Myositis specimens revealed a tendency towards more immature DC. MB modulated the maturation state of DC and DC recovered from MB-coculture had an inhibitory effect on T-cell proliferation. MB-lysates strongly stimulated MPh phagocytosis. Hypothetically, MB might modulate APC, counterbalancing immune-mediated damage.     

The nature of macrophages (foam cells) in neurinomas. Tissue culture study

Summary Fourteen cases of neurinomas of variable location are studied by tissue culture technic in an attempt to typify the foam cells as primary or superimposed elements in the tumor population. Our results demonstrate that the in vitro behavior of the neurinomas is constant and characteristic and that three cell types are found in them: fusiform, star-shaped cells and macrophages. There appears that the macrophage is an evolutive aspect of the star-shaped cells and probably of the fusiform one. On this basis, macrophage and foam cells of neurinomas must be considered as primary.     

Nephrogenic systemic fibrosis presenting as myopathy: A case report with histopathologic correlation

Nephrogenic systemic fibrosis is primarily a skin disorder associated with renal insufficiency and exposure to gadolinium-containing (GAD+) contrast. We present the case of a 64-year-old man who was exposed to gadolinium while in acute renal failure, and months later developed limb stiffness, proximal weakness, and woody muscle texture. Muscle biopsy demonstrated chronic non-inflammatory fibrosing myopathy. CD34+ fibroblasts have previously been reported to be specific for nephrogenic systemic fibrosis dermopathy, and we found these in fibrotic areas of muscle and fascia. Nephrogenic systemic fibrosis is an emerging disorder, and our case highlights that it may present as a progressive myopathy with minimal skin findings.     

Mineralized cells: Neurons,glia or macrophages?

Summary Mineralized dead cells were found in infarcted areas in the cerebella of three chicks killed in the healing stage of nutritional encephalopathy. The mineral deposits were found in vacuoles and consisted of radially packed threads and spicules, concentric laminated structures and centrally located amorphous masses. The cells in which these deposits were found were positively identified as astrocytes and macrophages. The presence of such deposits in neurons was suspected but not proven.Supported by Grant 1002 of the United States-Israel Bimational Foundation, Jerusalem, Isreal. M. W. is an established investigator of the Bureau of the Chief Scientist, Ministry of Health, Israel     

The cell-specific expression of metalloproteinase-disintegrins (ADAMs) in inflammatory myopathies

Inflammatory cell invasion and cytokine activation are important steps in the pathogenesis of immune-mediated diseases of muscle. Metalloproteinase-disintegrins (ADAMs) are considered to play a critical role in leukocyte migration by promoting cellular adhesion, cleavage of molecules of the extracellular matrix and shedding of membrane bound cytokines. Here, we report the expression patterns of ADAM8, ADAM9, ADAM10, ADAM12, ADAM17 and ADAM19 in cultured human myoblasts and peripheral blood mononuclear cells (PBMCs) in vitro, as well as in biopsies from patients suffering from polymyositis (PM), dermatomyositis (DM), inclusion body myositis (IBM) and non-inflammatory controls. We observed an in vitro downregulation of the RNAs of ADAM10, ADAM17 and ADAM19 in myoblasts after stimulation with various pro- and anti-inflammatory mediators, whereas in PBMCs an RNA upregulation of ADAM9, ADAM10, ADAM17 and ADAM19 was detectable under identical conditions. In human muscle biopsies, invading CD3+ T lymphocytes expressed ADAM17 and ADAM19, whereas macrophages co-localized to ADAM8, as detected by immunohistochemistry. Transfection of PBMCs with ADAM19 single interfering RNA and incubation with a metalloproteinase inhibitor suggest proteolytic activity of ADAM19 and involvement in the shedding of tumor necrosis factor-alpha. No differences in the cellular expression profiles between PM, DM and IBM were found, whereas the sections from non-inflammatory controls did not reveal any positive immunoreactivity for ADAMs, except for ADAM10, which is localized exclusively to muscle fibres. Our results suggest that certain ADAMs are expressed by specific cell populations during the genesis of immune-mediated diseases of human muscle.