多发性肌炎合并心脏损害的临床特点

目的　探讨多发性肌炎合并心脏损害的临床特点、诊断、治疗与预后。方法　回顾性分析41例多发性肌炎合并心脏损害患者的临床资料。结果　合并心脏损害者占同期全部多发性肌炎患者的38 3%,其中无症状者25例(60. 1% );平均年龄较无心脏损害者高(P<0 .05);出现发热、合并间质性肺病的比率高(P<0. 05);心电图异常主要表现为窦性心动过速(31.7% )、窦性心律不齐(26. 8% )。超声心动图异常者中以心包积液居多。血清抗核抗体(ANA)阳性率及C 反应蛋白含量明显增高(均P<0 .05)。对心脏损害采用病因及对症治疗,治愈14例,症状改善23例,恶化2例,死亡2例。结论　心脏损害是多发性肌炎最常见的并发症,大多数患者无自觉症状,心电图是诊断心脏损害的主要方法。严重的心脏损害是导致患者死亡的重要因素,及时发现心脏损害并采取有效治疗对于改善患者的预后有重要意义。     

多发性肌炎合并间质性肺病一例

多发性肌炎（PM）是一种肌肉炎性免疫性疾病，除累及肌肉外，还可引起肺脏、心脏及胃肠道等多系统损害。间质性肺病（ILD）是PM患者最常见、最严重的并发症，大部分病例抗Jo-1抗体阳性。     

神经性肌炎7例分析

目的:通过对7例多发性肌炎合并周围神经损害(神经性肌炎)患者临床资料的分析以探讨其临床特点。方法:采用回顾性研究方法。结果:神经性肌炎患者血清肌酶水平增高不明显,肌电图有神经性损害,肌肉病理为不典型炎症改变,激素及免疫抑制剂治疗效果不佳。结论:多发性肌炎合并周围神经损害(神经性肌炎)有特殊的临床特点,肌电图和肌活检对诊断有重要意义。     

特发性炎性肌病的相关特异性抗体的研究进展

特发性炎性肌病(IIM)主要包括多肌炎、皮肌炎和免疫性坏死性肌病等。近年来已在青少年和成人IIM中鉴定出不同的抗体,例如抗Jo-1抗体、抗NXP2抗体、抗MDA5抗体和抗HMGCR抗体等。这些抗体阳性的IIM可表现出不同的临床、病理表现,并且可以帮助临床医生区分更精确的亚型,从而指导治疗及评估预后。     

单纯多发性肌炎46例分析

目的总结单纯多发性肌炎临床特点,探讨实验室检查与临床症状及疗效的关系。方法综合分析住院的４６例单纯多发性肌炎的临床资料。结果全部患者有肢体近端肌无力;严重者,肌酶升高明显;自身抗体阳性者疗效较差。结论肌酶谱变化及自身抗体阳性与否对判断病情及疗效有重要价值。     

皮肌炎相关的肌炎特异性自身抗体研究进展

皮肌炎是自身免疫性疾病,以对称性四肢近端肌肉无力为特征性表现,通常伴有典型的皮肤损害,易侵犯多脏器、多系统,临床较复杂,且常伴发肿瘤。近期研究发现,皮肌炎患者血清中存在多种自身抗体,包括肌炎特异性自身抗体和肌炎相关性自身抗体,肌炎特异性自身抗体与某些临床表现密切相关,临床上通过对这些自身抗体检测可帮助疾病诊断及判断预后。     

多发性肌炎与“抗JO-1综合征”

近年研究证实,多发性肌炎(ＰＭ)和皮肌炎(ＤＭ)病人血清中可检测出抗组氨酰ｔＲＮＡ合成酶(Ｊｏ-1)抗体等多种“肌炎特异性自身抗体”,并发现抗体阳性病人常伴发间质性肺病(ＩＬＤ),关节炎和Ｒａｙｎａｕｄ现象等合并症,并称之为“抗Ｊｏ-1综合征”或“抗合成酶综合征”[1,2]。本文应用免疫印迹技术(ＩＢＴ)检测52例经临床、肌电图、肌活检和血清酶学确诊的ＰＭ和ＤＭ病人血清可提取性核抗原(ＥＮＡ多肽)抗体,着重分析Ｊｏ-1抗体阳性病人的临床特征及其并发症,并对本病的发病机理进行初步探讨。资　　料临床资料　ＰＭ22例,男5例、女17例,年…     

肌炎特异性自身抗体在多发性肌炎/皮肌炎及其他神经肌肉疾病的表达

目的 探讨肌炎特异性自身抗体(MSAs)在多发性肌炎/皮肌炎(PM/DM)及其他神经肌肉疾病的敏感度和特异度.方法 采用免疫斑点分析法测定63例PM/DM(PM/DM组)及60例神经肌肉疾病(非肌炎)患者(对照组)的血清抗Jo-1抗体和抗SRP抗体水平,分析其对PM/DM诊断的敏感度和特异度.结果 PM/DM组抗Jo-1和抗SRP抗体阳性率分别为17%和5%,对照组均为阴性.两种抗体诊断PM/DM特异度均为100%(95%CI:94%～100%),总敏感度为22%(95%CI:13%～34%).结论 抗Jo-1和抗SRP自身抗体对PM和DM特异度高.     

血清抗糖脂抗体在自身免疫性多发性周围神经病中的意义

目的 :探讨抗周围神经膜表面糖脂抗体在自身免疫介导的多发性周围神经病中的临床意义。方法 :ELISA法测定格林 巴利综合征和慢性炎性脱髓鞘性周围神经病患者血清中抗硫脂抗体和 7种节苷脂抗体 (GM1、GM2、GA1、GD1a、GD1b、GT1b和GQ1b)的阳性率。结果 :GBS和CIDP组患者抗硫脂抗体、抗GM1、GA1和GD1b抗体的阳性率显著高于非自身免疫性周围神经病组 (P <0 0 5 ) ;此两组患者间的抗体阳性率比较则无显著差异 ;GBS组内有眼 /咽肌麻痹的患者 (17例 )与无眼肌和 (或 )咽喉肌麻痹患者 (2 5例 )的节苷脂抗体阳性率也无显著差异。结论 :血清抗硫脂抗体和抗GM1抗体增高对自身免疫介导的多发性周围神经病患者有一定临床意义。     

抗Hu抗体检测在神经系统副肿瘤综合征诊断中的临床意义

目的 探讨抗Hu抗体检测在神经系统副肿瘤综合征诊断中的临床意义.方法 采用间接免疫荧光方法和蛋白免疫印迹法对送检至北京协和医院神经病理实验室的1500余例患者的血清和脑脊液进行抗Hu抗体检测,回顾性分析抗Hu抗体阳性患者的临床资料及诊断.神经系统副肿瘤综合征的诊断标准参照Graus等的诊断标准.结果 共有27例患者抗体阳性,其中血清抗Hu抗体阳性25例,脑脊液抗Hu抗体阳性8例.临床主要表现为感觉神经元神经病、亚急性小脑变性、Lambert-Eaton综合征和抗利尿激素分泌不当综合征引起的脑病等,其中20例(74.1%)患有肿瘤,包括肺癌17例,胃癌2例,不明性质腹部肿瘤1例.7例患者未发现恶性肿瘤,包括多发性肌炎和系统性红斑狼疮(SLE)合并神经肌肉病各1例.根据Graus等神经系统副肿瘤综合征的诊断标准,27例中22例可确诊神经系统副肿瘤综合征或相关的肿瘤,抗Hu抗体的阳性预测值为81.5%.结论 抗Hu抗体阳性对神经系统副肿瘤综合征的诊断具有一定意义;其相关肿瘤以肺癌,特别是小细胞肺癌最常见.其他自身免疫性疾病偶可见抗Hu抗体阳性,需要全面检查和密切随访以排除恶性肿瘤的可能.     

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。     

Clinical and pathologic features of focal myositis

To clarify the nosology of focal myositis (FM), we report the clinical and pathologic features of eight patients presenting with focal enlargement of one muscle. Most patients improved without immunosuppressive therapy, and none developed polymyositis. Pathologic features were those of an inflammatory myopathy, with muscle fiber hypertrophy and moderate to severe inflammation. In most cases, a clustering of tightly packed muscle fibers, enveloped by a thick bundle of fibrosis, was associated with the diagnosis of FM. Immunohistochemistry showed T cell predominance within the interstitial infiltrates in all cases. No evidence of vasculitis was present. Our findings suggest that FM is a benign condition that has certain clinical features separating it from other inflammatory myopathies. Pathologic changes, such as large clusters of nesting muscle fibers surrounded by thick fibrosis, are more characteristic of FM than polymyositis.     

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.     

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     

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。     

An autopsy case of polymyositis with exacerbation of chronic myocarditis]

A 38-year-old man had suffered from general fatigue, mild weakness of proximal muscles, and dry cough in November, 2000. Serum levels of muscle enzymes were elevated. Computed tomography of the chest revealed reticular appearance in the bilateral dorsal lung areas. He did not show any improvement, therefore he was referred to our hospital in April, 2001. He was diagnosed as mild polymyositis with mild interstitial pneumonia. He was treated intravenously with methylprednisolone pulse therapy. During the pulse therapy serum level of creatine kinase was decreased, but he died because of acute pump failure of the heart. The cause of the heart failure could be an exacerbation of chronic myocarditis associated with polymyositis and it was confirmed by autopsy findings. When a patient with mild polymyositis complains of general fatigue, myocarditis should be carefully evaluated because of the high risk of death.     

Inflammatory myopathies: evaluation and management

The inflammatory myopathies, including dermatomyositis, inclusion body myositis, and polymyositis, are poorly understood autoimmune diseases affecting skeletal muscle. Dermatomyositis is a disease mainly of skin and muscle, but may affect lung and other tissues. Proximal or generalized weakness or skin rash are the typical presenting features. Inclusion body myositis has a specific clinical pattern of weakness that generally distinguishes it from other inflammatory myopathies, with prominent involvement of wrist and finger flexors, and quadriceps. Polymyositis generally presents with proximal or generalized weakness. Typical dermatomyositis muscle pathology is quite distinct, with perivascular inflammatory cells that include plasmacytoid dendritic cells, and abnormal capillaries and perimysial perifascicular myofibers. Both inclusion body myositis and polymyositis usually have infiltration into muscle of large numbers of inflammatory cells, typically surrounding and displacing, and sometimes invading, myofibers. Inclusion body myositis is refractory to corticosteroids and to several immunomodulating therapies that have been used. Dermatomyositis and polymyositis are treated with corticosteroids and a variety of agents. Osteoporosis and opportunistic infections pose a significant risk during treatment of patients. This review discusses the clinical manifestations, pathology, and treatment approaches for the inflammatory myopathies.     

炎症性肌病247例临床与病理研究

目的探讨多发性肌炎（PM）、间质性肌炎（IM）与神经肌炎（NM）的临床特点及神经肌肉活检的诊断价值。方法回顾分析247例炎症性肌病的临床表现及神经肌肉活检结果。结果3组临床表现相似,为近端肌无力、肌痛等;均有不同程度的肌酶增高,但PM组增高明显;IM和NM具有PM的病理改变,但炎性程度不及PM,又各有其特异性,IM组为炎性细胞浸润间质,NM组神经活检多有髓鞘脱失、炎性细胞浸润。结论PM、IM、NM炎性肌病的临床表现相似,诊断困难,需结合神经肌肉活检等辅助检查才能作出正确的诊断。     

Dermatomyositis

Dermatomyositis is a condition that combines an inflammatory myopathy with characteristic cutaneous disease. This disorder is closely related to polymyositis, which has all the muscular features of dermatomyositits without the presence of skin disease. Both dermatomyositits and polymyositis may occur in the presence of other collagen vascular diseases such as lupus erythematosus, scleroderma, Sj?gren's syndrome, rheumatoid arthritis, and various vasculitides. Dermatomyositis seems to be characterized by an increased frequency of internal malignancy and both dermatomyositis and polymyositis are associated with morbidity and mortality. Therefore, prompt and aggressive therapy is necessary.