多发性肌炎和皮肌炎患者医院尿路感染分析

目的 探讨多发性肌炎(PM)和皮肌炎(DM)患者发生医院尿路感染的相关危险因素.方法 回顾性分析1996年3月至2006年3月在我科住院的115例PM和136例DM患者,记录其中出现尿路感染病例的临床资料.结果所有PM和DM患者中发生医院尿路感染者共21例(8.37%),感染菌以革兰氏阴性菌为主 (76.19%).激素(泼尼松)用量≥30 mg组医院尿路感染发生率明显高于激素用量＜30 mg组(9.62% vs 2.33%,P＜0.05);激素合并应用免疫抑制剂患者医院尿路感染发生率明显高于单用激素组(9.26% vs 2.86%,P＜0.05);留置导尿管患者医院尿路感染的发生率明显升高;另外,住院时间越长,肌酸肌酶水平越高,越易发生尿路感染.结论 PM和DM患者医院尿路感染的相关危险因素为性别、住院时间、激素及免疫抑制剂的应用、是否留置导尿管、肌酸肌酶水平等.     

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。     

Differential Immunohistological Features of Inflammatory Myopathies and Dysferlinopathy

This study was performed in order to characterize the types of the infiltrating cells, and the expression profiles of major histocompatibility complex (MHC) class I and membrane attack complex (MAC) in patients with inflammatory myopathies and dysferlinopathy. Immunohistochemical stains were performed using monoclonal antibodies against several inflammatory cell types, MHC class I, and MAC in muscles from inflammatory myopathies and dysferlinopathy. There was significant difference in the types of infiltrating cells between polymyositis (PM), dermatomyositis (DM), and dysferlinopathy, including significantly high CD4+/CD8+ T cell ratio and B/T cell ratio in DM. In dysferlinopathy, CD4+ T cells were the most abundant and the proportions of infiltrating cell types were similar to those of DM. MHC class I was expressed in muscle fibers of PM and DM regardless of the presence of inflammatory infiltrates. MAC was expressed in necrotic fibers and vessels of PM and DM. One patient with early stage DM had a MAC deposits on endomysial capillaries. In dysferlinopathy, MAC deposit was also observed on the sarcolemma of nonnecrotic fibers. The analysis of inflammatory cells, MHC class I expressions and MAC deposits may help to differentiate dysferlinopathy from idiopathic inflammatory myopathy.     

Sarcoid myositis with muscle weakness as a presenting symptom

A 54-year-old woman complaining of muscle weakness and weight loss was admitted to our hospital with suspected polymyositis. Muscle biopsy revealed Langhans-type giant cells and noncaseating granulomas. Therefore, sarcoid myositis was diagnosed. The patient was treated with prednisolone, and the symptoms improved gradually. Generally, sarcoidosis is identified clinically in patients with foggy vision or mediastinal lymphadenopathy, but muscular weakness may be an infrequently observed initial symptom. Sarcoidosis should be considered in the differential diagnosis of polymyositis.     

Infection-related morbidity and mortality in patients with connective tissue diseases: a systematic review

Patients suffering from connective tissue diseases (CTDs) constitute an important subgroup of immunosuppressed patients at risk for developing serious infections. Prophylactic antibiotic administration may decrease infection-related morbidity and mortality burden in patients with CTD, though one needs first to evaluate the overall effect of infection on morbidity and mortality in such patients and the presence of adequate prognostic/risk factors for infection development. Studies focusing on infection-related morbidity and mortality in patients with CTD were reviewed. Data on disease type, therapeutic regimens used, including corticosteroid dose and method of administration as well as other immunosuppressive agents, and outcome were extracted to evaluate the existence of specific treatment patterns predisposing to infection as well as infectious disease-related morbidity and mortality in patients with CTD. Thirty-nine studies focusing on infection incidence and/or outcome in patients with CTD were identified and analyzed; the majority of the reviewed studies (20) included patients with systemic lupus erythematosus (SLE). The mortality attributed to infection was 5.2%, while the overall mortality was 20%. There were no adequate data on the specific effect patterns of corticosteroid and immunosuppressant treatment on infection risk. Pneumocystis jiroveci (carinii) pneumonia, evaluated independently, exhibited significant mortality in patients with Wegener’s granulomatosis, polymyositis/dermatomyositis, and SLE. In conclusion, infectious diseases are a major cause of mortality in patients with CTD. However, treatment-related factors predisposing to serious infections have not been adequately outlined. In addition, there are no data regarding the effect of prophylactic practices involving antibiotic administration in morbidity and mortality.     

神经肌炎与多发性肌炎的对比分析

目的 :为了认识神经肌炎的临床、神经电生理等方面特点 ,探讨其与多发性肌炎的关系。方法 :选取我院近 10年间收治的较典型的 11例神经肌炎病人 ,随机选取同期收治的多发性肌炎病人 2 9例 ,对此两组病人的临床表现、心脏并发症、对激素治疗的疗效、肌电图以及肌肉活检的结果给予对比研究。结果 :在神经肌炎组中 ,有 8例感觉障碍 (6例呈末梢型感觉障碍分布 ;2例呈多发性神经根神经炎表现 ) ;神经传导速度检查中 ,10例有运动传导速度明显减慢或引不出 ,1例电位波幅明显降低 ,呈轴索样损害的表现 ;多发性肌炎组病人均无感觉障碍 ,神经传导速度检查均正常。两组在其他临床表现及激素治疗效果上 ,血清CK增高 ,肌活检、肌电图方面无明显差异(P >0 0 5 )。结论 :神经肌炎是多发性肌炎中一种少见的特殊类型 ,除具有多发性肌炎的表现之外 ,同时还具有周围神经病的表现 ,两者间是很难区分的     

Marked increase of serum hepatitis C virus (HCV) RNA titer during treatment with high-dose prednisolone in a case of polymyositis

A 70-year-old Japanese woman with hepatitis C virus (HCV) infection was diagnosed with polymyositis and treated with high-dose prednisolone (PSL). The serum alanine aminotransferase (ALT) level increased from 78 to 345 U/l 1 week after initiating treatment, although the polymyositis settled promptly. Furthermore, the serum HCV RNA level increased markedly from 110 to 850 kIU/ml 3 weeks after starting treatment. Previously, the patient had suffered an occlusion of the left branch of the retinal vein secondary to hyperviscosity syndrome resulting from Sjögrens syndrome and low-dose PSL treatment had been commenced. The serum HCV RNA and transaminase levels had not increased during this low-dose PSL treatment. Although intensive immunosuppression is necessary as an initial treatment of several collagen diseases including polymyositis, high-dose PSL therapy may markedly augment the serum HCV RNA level and therefore careful observation is necessary in HCV-infected patients.     

抗Jo-1抗体在多发性肌炎/皮肌炎中的临床研究

目的摇对69例多发性肌炎/皮肌炎(PM/MD)病人抗Jo-1抗体进行检测,了解抗Jo-1抗体阳性与PM/DM临床症状的相关性及对PM/DM的诊断价值。方法应用免疫印迹(IBT)法检测PM/DM病人中的抗Jo-1抗体,对抗Jo-1抗体阳性血清进行免疫双扩散(ID)检测,两者抗Jo-1抗体均阳性者为抗Jo-1抗体阳性组,其他为抗Jo-1抗体阴性组,前瞻性比较PM/DM抗Jo-1抗体阳性组和抗Jo-1抗体阴性组的临床特征。结果本组中抗Jo-1抗体阳性占32%,抗Jo-1抗体阳性患者出现肺间质病变(ILD)、多关节炎/多关节痛、肺部感染和雷诺现象分别为18例(82%)、18例(82%)、17例(77%)、7例(32%);与抗Jo-1抗体阴性组比较,差异有显著性(P<0.05)。另外,在阳性组出现吞咽困难1例(5%),而阴性组为15例(32%),两组相比差异有显著性(P<0郾05)。结论抗Jo-1抗体阳性组PM/DM患者的肺间质病变及多关节炎症状突出,合并肺部感染的概率高,而肌痛或肌无力症状较轻,出现吞咽困难症状概率较低;阴性组出现吞咽困难症状概率较阳性组高。     

Humoral aspects of polymyositis/dermatomyositis

Evidence of the involvement of systemic autoimmunity has been observed in polymyositis/dermatomyositis (PM/DM). Autoantibodies directed against various cellular constituents have been detected in most patients with PM/DM, and about one-third of patients have autoantibodies (myositis-specific antibodies: MSAs) that are found specifically in myositis patients. These autoantibodies are closely associated with a characteristic clinical subgroup, and therefore help in establishing the correct diagnosis, classifying the myositis patients in a homogeneous subset, and facilitating the clinical and treatment follow-up. Autoantibodies to six of the aminoacyl tRNA synthetases are each associated with a similar syndrome marked by myositis, interstitial lung disease, arthritis, and other features constituting an "antisynthetase syndrome." Antibodies to other cytoplasmic antigens that are involved in protein synthesis or translation factors are seen in a small proportion of patients. Antisignal recognition particles are associated with severe, refractory myositis that differs significantly from antisynthetase syndrome. Antibodies to the nuclear antigen are specifically seen in patietnts with DM. Several autoantibodies, including anti-U1 RNP, anti-U2 RNP, anti-Ku, and anti-PM-Scl, have been associated with scleroderma–PM overlap. In recent years, these MSAs and their antigens have been characterized using molecular biology approaches. It is not known if the MSAs are involved in tissue injury or the pathogenesis of PM/DM. However, an understanding of the production mechanisms of these autoantibodies can provide insight into the etiology of this disorder.