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.     

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。     

原发性多发性肌炎—皮肌炎几项指标对早期诊断的探讨

为达到早期诊断多发性肌炎、皮肌炎的目的,对我院45例住院确诊病人进行了病理、肌电图、自身抗体、酶学检查,以期发现临床与各项检查的相互关系及对早期诊断的价值。资料本组45例,多发性肌炎23例,皮肌炎22例。男17例,女28例。年龄:40岁以上者14例,39岁以下者31例。病程1个月至17年。半年以上者25例(55.5％)。首发症状为无力者39例 (86.6％)。病前有发热6例,肌痛及关节痛24例,有上感等前驱症状者37例(82.2％)。体征:①肌无力43例(95.5％),其中颈肌无力者5例,吞咽困     

特发性肌炎临床与病理分析

目的 研究特发性肌炎临床及肌活检病理的特点。方法 对86例多发性肌炎,41例皮肌炎惠的临床、肌电图、血清肌酶及经开放性手术获得肌活检标本的病理特点进行分析。结果 特发性肌炎：(1)近端肌无力88．9％,远端肌无力622％,吞咽困难37.8%,颈肌无力18．1％,肌痛276％。(2)肌活检病理：坏死63．8％,萎缩37．8％,再生354％,间质炎236％,束周萎缩11．8％。结论 多发性肌炎和皮肌炎临床症状、肌电图、血清肌酶等除皮损外,无很大差异,诊断特发性肌炎肌肉活检是重要手段;束周萎缩为皮肌炎的特征性的病理改变;肌痛虽是特发性肌炎较为特征临床表现,但仅见于部分患。     

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/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。     

吞咽困难的多发性肌炎与皮肌炎(附14例临床及1例病理报告)

多发性肌炎与皮肌炎,近年来国内报告渐多,但以吞咽困难为首发症状或主要症状者未见单独报告。我院曾收治多发性肌炎与皮肌炎共47例(多发性肌炎16例,皮肌炎31例),其中以吞咽困难为首发症状或主要症状者14例(多发性肌炎3例,皮肌炎11例)死亡5例,尸检1例,现报告如下。     

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、皮肌炎和包涵体肌炎

一般认为炎性肌病有三种类型,即多发性肌炎、皮肌炎和包涵体肌炎。不论其单独发生或与其他疾病同时存在,均保持其临床、免疫病理和形态学特征。三者的患病率约为1/10万。皮肌炎在儿童和成人中均可患病,女性较男性多见;而多发性肌炎出现在20岁以后,儿童少见。包涵体肌炎多发生在50岁以后,男性是女性的3倍,白人比黑人多见。三者的起病均与自身免疫有关,临床上的共同特征是进行性肌无力,一般远端受累较晚。但包涵体肌炎的远端受累较早,特别是足部伸肌、手指屈肌的受累,可为临床诊断的早期线索。     

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。     

Clinical study of 12 pathologically proven cases

Among 70 pathologically proven MS cases, we found 12 cases with onset before 25. Only females were affected. There was no positive familial history of MS. In every case the diagnosis was considered during life. Mean duration of the disease was 29.1 +/- 12.5 years. This was significantly longer (p less than 0.001) than in the control group (58 pathologically proven cases with onset after 25). There were no acute courses. Progressive courses (1 case) and remittent courses (5 cases) were less frequent than in the control group (p less than 0.001 and p less than 0.01, respectively). The frequency of remittent-progressive courses (6 cases) was similar in both groups. The length of remittent-progressive courses (30.2 +/- 16.2) and remittent courses (28.6 +/- 9.7) was longer than that of control group (p less than 0.001). The symptomatology of onset was similar in both groups and had no predictive value for prognosis. The overall frequency of signs and symptoms was not statistically different in both groups. For each sign, the percentage of affected patients, throughout the course of the disease, was always smaller in early onset MS than in control group, except for optic neuritis which was as frequent in both groups. However, this was statistically significant only for ocular motricity and nystagmus.     

Clinical analysis of 70 neuropathologic cases of multiple sclerosis

A retrospective study of clinical files of 70 pathologically confirmed cases of Multiple Sclerosis (MS) (53 women and 17 men), selected from the records of the Laboratoire de Neuropathologie Charles Foix (H?pital de la Salpêtrière) was performed. The following data were recorded and analysed by a computer program (HP 85): sex, age of onset of disease, clinical course (classified into Remittent, Remittent-Progressive, Progressive and Acute) and the date of each new neurological symptom or sign. The mean age of onset was 36.8 +/- 12. In women, the disease began earlier (34.6 +/- 12) and the duration was longer (17.4 +/- 12). In men the age onset was 40.6 +/- 11 and the duration was 12.5 +/- 6. In remittent courses, the mean age of onset was 30.8 +/- 13 and the duration was 21 +/- 10. In progressive courses, the age at onset was 45 +/- 10 and the duration was 2. In women, progressive courses began significantly later (42.3 +/- 9.2) and were shorter (15 +/- 8) than remittent courses which began at 26.8 +/- 8.2 and lasted 23 +/- 10. The histogram of the duration of clinical courses showed three groups: acute courses (8 cases less than 5 years long), intermediate courses (41 cases, between 5 and 20 years long), and long courses (21 cases longer than 20 years). Women were more often affected with acute (7/8 cases) or long courses (20/21 cases). The mean duration of the disease was the same when the symptoms and signs at onset were motor weakness, sensory disturbances, optic neuritis or diplopia.(ABSTRACT TRUNCATED AT 250 WORDS)     

貌似多发性肌炎的脂质沉积性肌病病理改变

目的：研究貌似多发性肌炎的脂质沉积性肌病的肌肉病理改变,以解释临床现象和探讨发生机制。方法：择取脂质沉积性肌病伴血清肌酶明显升高的 人14例,将临床表现,血清肌酶,肌电图和肌肉病理及肌肉组织化学改变进行总结分析。结果：临床上一般呈急性或亚急性起病,或呈反复发作性病程,肌无力明显,血清肌酶明显升高,激素治疗反应好,肌肉病理可见肌纤维内脂滴明显增多,形成HE染色可见的空泡,ORO染色证实肌纤维内有大量脂滴,其中4例可见个别坏死肌纤维和再生纤维,5例有较严重的肌纤维坏死,对其中3例反复肌肉活检,发现在脂质贮积的基础上,病程中的某一阶段发生明显的肌纤维坏死,其余病例虽有血清肌酶的明显升高,而病理上并未见到明显的肌纤维坏死,部分肌纤维非特异性酯 酶活性增高。结论：有些脂质沉积性肌病与多发性肌炎十分相似,需要借助肌肉病理和肌肉组织化学检查,甚至反复肌肉活检才能确定,肌纤维内脂肪代谢障碍造成肌纤维破坏和肌酶的外漏,可能与细胞内环境改变和细胞膜通过透性加有关。     

Quantitative histopathology of the inflammatory myopathies

This study quantitatively assessed skeletal muscle histopathology in 57 patients with inflammatory myopathy, including 20 patients with polymyositis (PM), 19 patients with dermatomyositis (DM), and 18 patients with evidence of an additional connective-tissue disease. No histologic criteria for invariably distinguishing patients with inflammatory myopathy were established because of overlap in individual measurements, but general histopathologic distinctions were confirmed. In PM, endomysial mononuclear cell infiltration (fibers bordering on inflammation) was usual, whereas in DM inflammation of large vessels, fibers with circumscribed areas of myofibrillar loss, and perifascicular atrophy were seen. Patients with evidence of an additional connective-tissue disease were most similar to the DM patients, with a greater prevalence of perivascular inflammation than in the PM patients. Because of varying histopathology (and presumed varying pathogenesis), future therapeutic trials would be more informative if they were designed using patients with homogeneous histologic features.     

强直性肌营养不良临床特点分析

目的分析强直性肌营养不良(DM)的临床特点,以提高对DM疾病的认识及诊断水平。方法对21例DM患者的临床资料进行回顾性总结与分析。结果 21例患者均为慢性起病,以双手无力,活动不灵活起病多见,其中5例有家族史,部分病例伴有心脏、眼部、内分泌及中枢神经系统等其他多系统损害。19例行肌电图检查提示肌源性损害,其中16例发现有肌强直电位。10例行肌活检,主要表现为部分肌纤维萎缩,变性、坏死肌纤维,核内移及肌浆块形成,部分萎缩纤维内可见无结构胞浆体。1例强直性肌营养不良蛋白激酶(DMPK)基因CTG重复序列分析发现拷贝数超过正常范围。结论 DM是一种主要累及肌肉系统,以肌强直、肌无力和肌萎缩为主要临床表现并伴有多系统损害的疾病。综合评估多系统损害并结合肌肉的电生理学及病理学检查,有助于提高对DM的认识;在有条件的医疗机构可以开展DM基因诊断,对DM确诊很有意义。     

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.     

皮肌炎肌组织中浆细胞样树突状细胞的病理学特点

目的 研究皮肌炎肌组织中浆细胞样树突状细胞(pDC)的病理学特点,探讨其病理学意义.方法 收集30例皮肌炎及25例多发性肌炎(PM)患者的肌肉活体组织标本,全部行HE染色、免疫组织化学(抗CD303分子及抗CD68分子)染色.结果 肌活体组织检查病理示,30例皮肌炎中25例可见典型束周萎缩,伴肌束膜明显增宽,5例束周萎缩不典型,但于肌束周边可见小群坏死或再生纤维;14例可见束周凿空样纤维;17例可见肌束膜内血管周围炎,6例于肌内膜可见灶性炎症细胞浸润.免疫组织化学染色示,19例在肌束膜及血管周围可见较多巨噬细胞浸润;20例可见较多pDC浸润,其中,15例此细胞仅位于宽大肌束膜内血管周围的灶性炎细胞区域,3例仅位于肌内膜灶性炎症细胞区域,2例pDC在上述2个部位均存在.25例PM患者的主要病理改变为坏死、再生纤维及炎症细胞浸润,免疫组织化学染色在血管周围灶性炎症细胞区域未发现或偶见少量散在pDC的浸润.结论 在PM患者肌组织中未发现或偶见少量散在pDC的浸润,而在皮肌炎患者肌组织内可见显著pDC的浸润,且其主要在宽大肌束膜内的血管周围分布的特点提示可能与皮肌炎束周萎缩的形成有关.

Abstract：

Objective To study the histological features of plasmacytoid dendritic cells (pDC) in muscle tissue affected by dermatomyositis (DM) and to discuss the pathological significations of pDC.Methods Muscle tissues from 30 cases of DM and 25 cases of polymyositis (PM) were collected.HE stain, immunohistochemistry studies were carried out in all muscle samples.Results Pathological features of DM included: perifascicular atrophy (25/30); punched-out fiber (14/30); perivasculitis (17/30),inflammatory infiltration in the endomysium(6/30).Using immunohistochemistry study, 19 cases from DM were infiltrated by macrophages which are CD68 positive and CD303 negative, 20 cases with DM were infiltrated by pDC which are CD303 positive.The location of pDC were: perivascular of interfascicular septae only (15/20); endomysium only (3/20) and both (2/20).Myopathic damage such as necrotic and regenerating fibers and inflammatory infiltration could be seen in PM.There was few pDC infiltration in PM.Conclusions There is few pDC in muscle tissue affected by PM and many pDC in muscle tissue affected by DM with infiltration mainly in the wide interfascicular septae.pDC may be connected to perifascicular atrophy and play a roll in the pathogenesis of DM.     

135 cases of polymyositis

The classification of 135 patients with either dermatomyositis (DM) or polymyositis (PM) showed 34 cases of DM and 56 cases of PM in adults, 6 cases of DM associated with cancer, 9 cases of DM in children, 16 cases of localized PM and 14 cases of an overlapping syndrome. Results of biological tests, erythrocyte sedimentation rate and serum enzyme determinations were inconstantly abnormal. The electromyograms were generally of a myogenic type with spontaneous activity in about half the cases. Muscle biopsy usually showed inflammation necrosis and regeneration, sometimes only of moderate severity only. Results were normal in several cases. In 21 patients only the pathognomonic perifascicular atrophy was reported. Proposed classifications are unsatisfactory. Polymyositis may be considered as a syndrome. Among the primary forms the distinction between acute dermatomyositis and subacute or chronic polymyositis is poorly defined and passage from one disorder to another is frequent. Pseudo-myasthenic forms are not entities and pseudo-myopathic types are actually muscular dystrophies. Associations with polymyositis are common and may consist only in the addition of one sign of no clinical significance (PM "plus"). The polymyositis lesion may be part of a syndrome such as Gougerot-Sj?gren's or of another connective tissue disease. A system of diagnostic criteria uses numerical ratings of each criterion as a function of its semiologic importance.