特发性炎性肌病及合并间质性肺病的自身抗体研究进展

特发性炎性肌病(IIM)是以四肢近端肌肉受累为主要临床表现的自身免疫性疾病,可分为5种类型:多发性肌炎、皮肌炎、儿童多发性肌炎/皮肌炎、皮肌炎合并恶性肿瘤以及包涵体肌炎,临床上以多发性肌炎和皮肌炎最常见.IIM患者的自身抗体与某些临床表现密切相关,自身免疫功能异常是IIM发生发展的关键.     

多发性肌炎和皮肌炎(附13例分析)

多发性肌炎和皮肌炎是以肌无力和肌痛为主要症状,以皮肤肌肉炎症及坏死性病理变化为特征的一组疾病。本病临床诊断比较困难。现将我院收治的皮肌炎8例、多肌炎5例报告如下: 资料分析本组13例均无遗传史,也无肿瘤及其他结     

多发性肌炎/皮肌炎的神经肌肉活检术病理诊断

目的确定病理诊断多发性肌炎/皮肌炎的神经肌肉活检方法。方法对30例多发性肌炎/皮肌炎患者的临床表现及实验室资料进行回顾性分析。结果多发性肌炎的炎性细胞散布于整个肌肉并集中于肌纤维膜和肌内膜,而皮肌炎表现为束周肌纤维萎缩的特征,炎性细胞浸润主要在肌束膜的结缔组织。结论神经肌肉活检术对病理学诊断多发性肌炎/皮肌炎具有重要意义。     

女性多发性肌炎及皮肌炎患者骨密度相关因素分析

目的探讨女性多发性肌炎及皮肌炎患者骨密度的变化和骨质疏松的发生情况，并讨论疾病的病情和激素用量对骨密度的影响。方法采用双能X线骨密度仪分别测量36例女性多发性肌炎及皮肌炎患者（多发性肌炎及皮肌炎组）和健康女性（对照组）的股骨颈、三角区、大转子及左前臂桡骨远端的骨密度，并同时记录多发性肌炎及皮肌炎患者的症状变化、临床指标、激素使用情况。对多发性肌炎及皮肌炎患者诸多影响骨密度的因素进行统计学分析。结果多发性肌炎及皮肌炎组中骨量减少及骨质疏松的发生率高于对照组（P〈0．05）。多发性肌炎及皮肌炎组中骨量异常的患者比骨量正常的患者年龄更大、病程更长、激素使用时间更长、激素累积用量更大（P〈0．05）。与非闭经妇女相比，闭经妇女出现骨质疏松的比例增加（P〈0.05）。多元线性回归分析发现激素的使用时间、激素的累积剂量与股骨颈骨密度值有线性回归关系。结论多发性肌炎及皮肌炎患者骨质疏松的发生率较正常人群明显增高，闭经妇女更易出现骨质疏松，激素的使用时间及累积剂量是影响骨密度的重要因素。     

特发性炎性肌病免疫学分组的初步研究

特发性炎性肌病(IIM)包含一大组病因、发病机制、临床特征、预后等不完全相同的异质性疾病。临床上常见的类型有多发性肌炎(PM)和皮肌炎(DM)。因IIM患者起病方式、临床表现、实验室检查等方面变化很大,给患者的诊断、治疗和预后判断造成很大困难,需要对其进行合理分类。虽然现有的诊断和分类标准对IIM的诊断、治疗和预后有一定的指导作用。但近年发现,这些方法区分的各类型IIM患者在临床、实验室、遗传学等方面差别不显著,而肌炎特异性抗体(MSA)与某些临床表现密切相关,有更好的分类作用。现将我院自1989年以来治疗的抗J_(0-1)阳性和阴性IIM患者进行分析,以提高对IIM异质性的认识,并对抗J_(0-1)抗体的疾病分类价值作初步探讨。     

多发性肌炎/皮肌炎肺损害14例临床分析

目的提高对多发性肌炎/皮肌炎并肺损害的认识。方法对14例多发性肌炎/皮肌炎合并肺损害患者的临床资料进行回顾性分析。结果36例多发性肌炎/皮肌炎患者中14例合并肺损害,占38.9%,临床主要表现为咳嗽、气短、发热。全部患者均给予大剂量的强的松联合甲氨蝶呤、环磷酰胺等免疫抑制剂长程治疗,症状明显改善8例,好转4例,无效2例。结论多发性肌炎/皮肌炎易并发肺部感染,皮质激素合并免疫抑制剂能暂时改善症状,但其预后不佳,病症率高。     

28例多发性(皮)肌炎临床肌电图及肌活检分析

多发性肌炎是以肌肉炎症性改变为特征的肌病 ,合并有皮肤病损时 ,称为皮肌炎 ,均属特发性炎性肌病。现将我们 1993年 7月至 1999年 3月 ,对2 8例多发性 (皮 )肌炎病人临床、肌电图、肌活检结果报告如下 ,并对病因及诊断加以讨论。1　临床资料1 1　一般资料　男 12例 ,女 16例 ;年龄 18～ 65岁。单纯型肌炎 11例 ,皮肌炎 13例 ,多发性 (皮 )肌炎重叠综合征 4例。病程 2 0ｄ至 5年 ,半年以内 14例 ,1年以内 8例 ,2～ 5年 6例。病前有前驱症状者 13例 ,其中上呼吸道感染发热者 7例 ,肺部感染 2例 ,皮疹 2例 ,关节痛 2例。1 2　临床表现　以肌无…     

多发性肌炎,皮肌炎45例临床与辅助检查研究

报道45例多发性肌炎、皮肌炎，其中多发性肌炎24例，皮肌炎21例。结合文献讨论了本病的临床表现、肌酶谱、尿肌酸和肌酐、肌电图、肌活检与肌肉CT扫描检查在诊断中的意义，提出开展肌肉CT扫描为本病的诊断提供了一个新的辅助手段。     

特发性肌炎的诊治体会

特发性炎性肌病（IIM）属于一种免疫性疾病。其基本的病变是横纹肌的变性以及坏死等,同时伴有浸润性的炎性细胞生长。IIM主要包括多发性肌炎（PM）、皮肌炎（DM）和包涵体肌炎（IBM）三种类型^[1]。IIM发病机制经过临床的不断探究,已经获得了较大的进步。其诊断标准以及药物的治疗方面仍然未得到较为完善的统一。近来由于生物制剂的逐渐发展,IIM的治疗再次获得较大的期待。     

关于多发性肌炎和皮肌炎的神经系统损害

关于多发性肌炎和皮肌炎的神经系统损害山西省人民医院（０３００１２）申高梅党雁华王淑英山西医科大学郝斌阳曲县人民医院李健民对多发性肌炎（ＰＭ）病人临床上的神经系统损害常被忽视。近年来，有人提出ＰＭ可呈现神经肌病的损害形式，但深入研究的不多。作者根据临床...     

The management of dermatomyositis: current treatment options

Dermatomyositis is traditionally classified as one of the idiopathic inflammatory myopathies. The traditional approach to the management of patients with dermatomyositis focuses predominantly on end points related to the systemic manifestations of this disorder, especially proximal muscle weakness resulting from myositis. However, the primary and secondary skin changes that are characteristic of dermatomyositis can, in themselves, produce significant morbidity and disability. This article presents a dermatological perspective on the management of dermatomyositis. As dermatological management approaches can vary between countries, an effort has been made to present a consensus dermatological view concerning this subject. A US dermatologist is most likely to see patients having dermatomyositis skin lesions in the following four clinical settings: soon after the onset of dermatomyositis skin disease activity before the appearance of clinically-evident muscle disease (i.e., ‘pre-myopathic’ dermatomyositis); during the course of clinically-amyopathic dermatomyositis; during co-management of classical dermatomyositis with other physicians; and for recrudescent skin disease activity in classical dermatomyositis patients after their symptomatic muscle inflammation has been fully suppressed by systematic immunosuppressive/immunomodulatory treatment (i.e., ‘postmyopathic’ dermatomyositis). Both topical and systemic therapies for dermatomyositis skin lesions encountered in these various settings will be discussed. In addition to specific approaches to the treatment of dermatomyositis skin lesions, broader management issues that dermatologists must be aware of when caring for dermatomyositis patients are included in this discussion (e.g., patient education, risk of associated systemic disease, such as myositis and interstitial lung disease, risk for occult malignancy, and prognosis counselling).     

The management of dermatomyositis: current treatment options

Dermatomyositis is traditionally classified as one of the idiopathic inflammatory myopathies. The traditional approach to the management of patients with dermatomyositis focuses predominantly on end points related to the systemic manifestations of this disorder, especially proximal muscle weakness resulting from myositis. However, the primary and secondary skin changes that are characteristic of dermatomyositis can, in themselves, produce significant morbidity and disability. This article presents a dermatological perspective on the management of dermatomyositis. As dermatological management approaches can vary between countries, an effort has been made to present a consensus dermatological view concerning this subject. A US dermatologist is most likely to see patients having dermatomyositis skin lesions in the following four clinical settings: soon after the onset of dermatomyositis skin disease activity before the appearance of clinically-evident muscle disease (i.e., 'pre-myopathic' dermatomyositis); during the course of clinically-amyopathic dermatomyositis; during co-management of classical dermatomyositis with other physicians; and for recrudescent skin disease activity in classical dermatomyositis patients after their symptomatic muscle inflammation has been fully suppressed by systematic immunosuppressive/immunomodulatory treatment (i.e., 'postmyopathic' dermatomyositis). Both topical and systemic therapies for dermatomyositis skin lesions encountered in these various settings will be discussed. In addition to specific approaches to the treatment of dermatomyositis skin lesions, broader management issues that dermatologists must be aware of when caring for dermatomyositis patients are included in this discussion (e.g., patient education, risk of associated systemic disease, such as myositis and interstitial lung disease, risk for occult malignancy, and prognosis counselling).     

Current management of polymyositis and dermatomyositis

This article reviews the clinical features and emphasises the treatment of polymyositis and dermatomyositis. Pharmacological intervention with corticosteroids and immunosuppressive agents is discussed. In addition, strategies regarding the initiation and subsequent tapering of these drugs are provided, and an algorithmic approach to the management of myositis is provided. Therapeutic modalities for patients with refractory disease are considered, and the potential adverse effects of such treatment are discussed. Since patients with myositis often have disease-related complaints other than muscle weakness, a practical treatment approach for these problems is also outlined.     

Treatment of the inflammatory myopathies: update and practical recommendations

Background: The inflammatory myopathies are a heterogeneous group of diseases including dermatomyositis, polymyositis, and inclusion body myositis. Clinical trials in myositis are rare, making it difficult to make clear recommendations on the treatment of these rare disorders. Objective: To give an overview of treatment options and strategies and to provide the clinician with a framework that can be used in treating patients with myositis. Methods: Results of clinical trials in myositis, case series and important case reports are presented and discussed. Results/conclusion: Most patients with dermatomyositis or polymyositis require treatment with oral high-dose prednisone combined with azathioprine or methotrexate to facilitate early tapering of prednisone. In case of treatment failure, intravenous immunoglobulin can be tried, followed by rituximab, mycophenolate mofetil, or tacrolimus depending on the specific clinical situation. A treatment trial with oral prednisone combined with methotrexate is advised in a subgroup of patients with inclusion body myositis.     

Review article: the gastrointestinal complications of myositis

Aliment Pharmacol Ther 31 , 359–365

Summary

Background The inflammatory myopathies are a group of acquired diseases characterized by a proximal myopathy caused by an inflammatory infiltrate of the skeletal muscle. The three major diseases are dermatomyositis, polymyositis and inclusion body myositis. Aims To review the gastrointestinal manifestations of myositis. Methods Over 110 articles in the English literature were reviewed. Results Dysphagia to solids and liquids occurs in patients with myositis. The pharyngo‐oesophageal muscle tone is lost and therefore patients develop nasal speech, hoarseness, nasal regurgitation and aspiration pneumonia. There is tongue weakness, flaccid vocal cords, poor palatal motion and pooling of secretions in the distended hypopharynx. Proximal oesophageal skeletal muscle dysfunction is demonstrated by manometry with low amplitude/absent pharyngeal contractions and decreased upper oesophageal sphincter pressures. Patients exhibit markedly elevated creatine kinase and lactate dehydrogenase levels consistent with muscle injury. Myositis can be associated with inflammatory bowel disease, coeliac disease and interferon treatment of hepatitis C. Corticosteroids and other immunosuppressive drugs comprise the mainstay of treatment. Inclusion body myositis responds poorly to these agents and therefore a myotomy is usually indicated. Conclusion Myositis mainly involves the skeletal muscles in the upper oesophagus with dysphagia, along with proximal muscle weakness.     

难治性皮肌炎的治疗研究进展

皮肌炎是一种特发性炎症性的累及皮肤或肌肉系统的疾病,具有特征性的皮肤表现和（或）对称性四肢近端肌肉无力症状。糖皮质激素和免疫抑制剂是控制该病的主要药物,但仍有许多病人经上述治疗无效,发展为难治性皮肌炎。该研究就生物制剂、缓释促肾上腺皮质激素注射剂、干细胞移植治疗及JAK抑制剂在难治性皮肌炎中的应用进展进行综述,以期为难治性皮肌炎的临床治疗提供更多参考。     

The molecular and cellular pathology of inflammatory muscle diseases.

The inflammatory muscle diseases dermatomyositis, polymyositis and inclusion body myositis are of unknown cause, but immune mechanisms are strongly implicated. Progress in the past two years has led to an improved understanding of the main molecular events involved in the immunological synapse between muscle and autoinvasive T cells. In particular, we now have a better understanding of TCR gene rearrangement in endomysial T cells, regulation of MHC expression, activity of co-stimulatory molecules, and the signalling cascades activated by cytokines, chemokines and metalloproteinases. Recent reports of an upregulation of strong anti-apoptotic molecules on the surface of muscle fibers identifies the end result of these disease processes, loss of muscle cells, as through necrosis, and not apoptosis. Such progress in molecular immunopathology has generated the interest to apply semispecific immunotherapies with the hope of halting disease progression or improving the strength of patients unresponsive to currently available non-specific immunotherapeutic interventions.     

Current and new targets for treating myositis

As treatment of refractory idiopathic inflammatory myopathies (IIM) has been challenging, there is growing interest in assessing new therapies that target various pathways implicated in the pathogenesis of IIM. In the largest clinical trial to date, rituximab was studied in adult and juvenile myositis, but the primary outcome was not met despite 83 percent of subjects with refractory myositis meeting the definition of improvement. The U.S. Food and Drug Administration (FDA) has recently granted approval to Octagam 10% immune globulin intravenous (IVIg), for the treatment of adult dermatomyositis based on impressive results from a double-blind placebo-controlled trial. Anti-tumor necrosis factor (anti-TNF) utility in IIM is not recommended and recent reports suggest this therapy may induce systemic autoimmune disease including myositis. Further, anti-IL6 therapy cannot be recommended as a recent trial of tocilizumab failed to reach its primary endpoint.Further studies are needed to assess the role of newer therapies such as abatacept (inhibition of T cell co-stimulation), sifalimumab (anti-IFNα), Janus kinase [JAK] inhibitors, apremilast (phosphodiesterase 4 inhibitor), and KZR-616 (selective inhibitor of the immunoproteasome) given their biological plausibility and encouraging recent small-case series results. The future of IIM therapy will depend on exploring biomarkers implicated in the etiopathogenesis of IIM, improvements in myositis classification based on serological and histopathological features, and well-designed controlled clinical trials using validated consensus outcome measures.     

Recent advances in the management of adult myositis

Standard drug therapy of adult polymyositis, dermatomyositis and inclusion body myositis includes high-dose corticosteroids and cytotoxic drugs (methotrexate, azathioprine (AZA) and cyclophosphamide). Recent data are in favour of the early introduction of a cytotoxic or immunomodulating drug in addition to corticosteroid therapy. In patients with corticosteroid- and cytotoxic-resistant myositis, promising novel approaches to management include: iv. megadose pulse methylprednisolone combined with cytotoxic drugs, combination therapy with both methotrexate and AZA, cyclosporin, tacrolimus, fludarabine and iv. immunoglobulin (IVIG). Recent advances in the understanding of the role of cytokines and complement, in the pathogenesis of myositis, have led to preliminary therapeutic trials of three biological agents: etanercept, infliximab and anti-C5 monoclonal antibody.     

多发性肌炎和皮肌炎临床特点分析

目的:对比分析多发性肌炎(PM)和皮肌炎(DM)的临床特征,提高临床认识、改善预后。方法:收集2006年8月至2009年8月间住院的PM和DM患者共32例,对其年龄、住院时间、肌力、血清肌酸磷酸激酶(CPK)水平、红细胞沉降率(ESR)、C反应蛋白(CRP)和股四头肌MRI、肺高分辩率CT(HRCT)进行比较分析。结果:DM组和PM组患者在年龄、肱二头肌力、股四头肌力、住院时间、ESR、CRP方面差异均无统计学意义(P>0.05)。CPK水平PM组(3032±2973)U/L显著高于DM组(390±947)U/L,差异有统计学意义(P=0.015)。DM和PM患者颈肌受累、吞咽困难、声音嘶哑等方面,差异无统计学意义(P>0.05)。DM组间质性肺炎(ILD)的发生率显著高于PM组患者(P=0.021),且MRI上水肿信号的改变比PM患者明显。PM组对糖皮质激素疗效显著优于DM组(P=0.013)。4例死亡均为DM患者,3例死于间质性肺炎合并感染后呼吸衰竭,1例死于横纹肌溶解综合症致急性肾功能衰竭。结论:DM患者CPK水平不及PM患者高,但并发ILD较多,预后较PM差。