皮肌炎/多发性肌炎伴发恶性肿瘤22例临床分析

目的研究皮肌炎(dermatomyositis,DM)/多发性肌炎(polymyositis,PM)伴发恶性肿瘤的临床相关因素。方法回顾性分析我院8年来收治的22例DM/PM合并恶性肿瘤患者的临床相关资料,统计其合并恶性肿瘤的种类,与DM/PM发病的先后顺序及时间关系,并对患者进行年龄、性别及相关实验室检查结果的比较。结果DM/PM合并恶性肿瘤发生率为12.29%,肿瘤类型多样,以肺癌、胃癌、鼻咽癌等为主。40岁以上年龄组DM/PM患者合并恶性肿瘤的危险性高于40岁以下年龄组。伴发恶性肿瘤的DM/PM患者ESR、AST、CK、LDH及ALP的水平较不伴发恶性肿瘤患者高。结论年龄大于40岁,ESR、CK等升高为DM/PM伴发恶性肿瘤的临床相关因素。     

多发性肌炎/皮肌炎患者外周血单一核细胞热休克蛋白90α,βmRNA的表达

多发性肌炎/皮肌炎(PM/DM)是一种严重威胁患者心身健康的自身免疫性疾病,其发病机制尚未阐明.糖皮质激素仍是目前最常应用的治疗药物,但在临床上约有20%的PM/DM患者对糖皮质激素治疗不敏感,甚至抵抗[1].     

多发性肌炎/皮肌炎患者外周血单一核细胞热休克蛋白90α,βmRNA的表达

多发性肌炎/皮肌炎(PM/DM)是一种严重威胁患者心身健康的自身免疫性疾病,其发病机制尚未阐明.糖皮质激素仍是目前最常应用的治疗药物,但在临床上约有20%的PM/DM患者对糖皮质激素治疗不敏感,甚至抵抗[1].     

多发性肌炎/皮肌炎患者血浆三种神经肽的检测及意义

多发性肌炎/皮肌炎(PM/DM)是一种病因未明的自身免疫性疾病,细胞免疫和体液免疫在其发病中起重要作用。近来研究发现,神经内分泌系统对免疫系统亦有重要的调节作用^[1]。神经肽Y、β-内啡肽和降钙基因相关肽(CGRP)作为重要的神经肽,与一些自身免疫性疾病的关系已有报道。我们检测了28例PM/DM患者血浆中神经肽Y、β-内啡肽和CGRP,以探讨这3种神经肽与PM/DM发病的关系。     

肌炎抗体与皮肌炎/多发性肌炎并发间质性肺疾病与恶性肿瘤的相关性分析

目的探索14种肌炎抗体与皮肌炎(DM)/多发性肌炎(PM)并发间质性肺疾病(ILD)和恶性肿瘤的相关性。方法采用免疫印迹法筛选149例DM/PM患者的14种肌炎抗体,并对其临床资料进行回顾性分析。结果 149例患者中DM 145例,PM 4例。男46例,女103例。高分辨CT诊断显示有71例(47.7%)诊断为ILD,78例(52.3%)未诊断ILD。DM/PM患者中发现恶性肿瘤15例,发生率为10.1%。通过二元Logistic回归分析发现,女性、MDA-5抗体及Ro-52抗体是DM/PM发生ILD的危险因素;Ro-52抗体对DM/PM并发ILD的影响仅次于MDA-5抗体;高龄及TIF1-γ抗体是DM/PM患者并发恶性肿瘤的危险因素。结论女性、MDA-5抗体及Ro-52抗体是DM/PM并发ILD的危险因素。高龄及TIF1-γ抗体是DM并发恶性肿瘤的危险因素。     

皮肌炎/多发性肌炎患者伴发恶性肿瘤危险因素的研究与Logistic回归分析

目的探讨皮肌炎/多发性肌炎(DM/PM)患者伴发肿瘤的危险因素及两者间的关系。方法对DM/PM伴发恶性肿瘤者的临床表现、实验室辅助检查指标和治疗情况进行单因素和Logistic回归分析。结果恶性肿瘤年龄偏大者多见,好发于DM后1~2年,PM后1~5年。DM伴发恶性肿瘤的危险因素有中度日光性皮炎和/或皮肤瘙痒、中度咽喉部肌群受累(如吞咽困难、声嘶等)、重度颈部肌群受累(如抬头困难)、消瘦等;而PM则为轻度呼吸肌受累(如呼吸困难)。结论对出现中度日光性皮炎和/或皮肤瘙痒、消瘦、中度咽喉部肌群、重度颈部肌群和轻度呼吸肌受累的中老年DM/PM者尤其在发病2~5年内应警惕伴发的恶性肿瘤。     

皮肌炎/多发性肌炎合并肺间质病变33例临床分析

目的研究皮肌炎或多发性肌炎(DM/PM)合并肺间质病变(ILD)的临床特点。方法对比分析116例DM/PM和33例DM/PM合并肺间质病变的临床表现、实验室检查。结果合并ILD组关节痛、发热等比无ILD组多见,差异有显著性或差异高度显著性;合并ILD组眼睑紫红色斑比无ILD组少见,抗Jo-1阳性率、ESR升高等比无ILD组多见,差异均有显著性。结论ILD在DM/PM中发病率较高,发热、关节痛、抗Jo-1阳性率、ESR升高是ILD发生的指标。     

DM/PM中核因子-κB的表达及作用机制探讨

目的 通过研究NF-κB在DM/PM患者骨骼肌的表达情况,以及DM患者血清体外对脐静脉内皮细胞(HUVEC)NF-κB表达的影响,初步探讨NF-κB在DM/PM中的相关作用的机制.方法 采用免疫组织化法对25例DM/PM患者骨骼肌中的NF-κB(P65)进行检测,取16例DM患者治疗前和6例治疗后的血清加入体外培养的HUVEC中,用细胞免疫组织化法检测HUVEC株NF-κB(P65)的表达.结果 NF-κB(P65)在DM/PM患者骨骼肌内的微血管内皮细胞和肌纤维中表达上调,与正常对照组相比差异有显著性(P＜0.05),正常对照组无表达;DM患者治疗前血清体外作用HUVEC 6h后,(11.126±1.920)％细胞表达阳性,24h后细胞的阳性率上升到(17.048±1.140)％,差异有统计学意义(P＜0.01);治疗后血清对脐静脉内皮细胞(HU-VEC)的NF-KB(P65)阳性表达率分别为(6.797±0.609)％(6h)和(11.273±0.643)％ (24h),二者均低于相应的治疗前表达率(P＜0.01).结论 NF-κB在DM/PM肌肉微血管肌纤维的炎症性反应过程中发挥重要作用,其在DM患者血清中可能有激活NF-κB的成分.     

皮肌炎/多发性肌炎病因及发病机制的研究进展

皮肌炎(dermatomyositis,DM)和多发性肌炎(polymyositis,PM)是一组主要累及皮肤和横纹肌的自身免疫性皮肤病。目前确切病因尚不清楚,认为可能与遗传、感染、恶性肿瘤和免疫因素等相关。近年来,随着对该病遗传学研究的不断深入,对其发病机制有了更进一步的认识,本文将重点从遗传学方面对DM/PM的病因及发病机制的进展做一综述。     

系统性红斑狼疮 皮肌炎和系统性硬皮病伴发脂膜炎26例临床分析

目的　探讨系统性红斑狼疮 (SLE)、皮肌炎 (DM)和系统性硬皮病 (PSS)伴发脂膜炎的临床、免疫、病理学特征、发病机理及治疗。方法　对 19例SLE、6例DM和 1例PSS伴发脂膜炎者的有关资料进行分析。结果　脂膜炎发生率为 2 .7% ,其中SLE2 .6% ,DM 5.3 % ,PSS1.1%。表现为单发或多发痛性皮下结节、斑块和溃疡 ,多见于四肢(57.7% )。组织病理学改变为脂肪小叶坏死、间质血管炎、栓塞形成及炎性细胞浸润。经皮质类固醇 +免疫抑制剂等治疗 ,可有明显改善。 3例分别死于败血症和成人呼吸窘迫综合征。结论　脂膜炎为SLE、DM和PSS少见的并发症 ;以发热、痛性皮下结节和溃疡为其特征 ;病理以脂肪小叶坏死为主 ,血管炎及栓塞可能是其发病的重要因素 ;皮质类固醇联合免疫抑制剂治疗有效     

Long‐term outcome of patients with polymyositis/ dermatomyositis and anti‐PM‐Scl antibody

Background To date, no series has analysed long‐term outcome in patients with polymyositis/dermatomyositis (PM/DM) with anti‐PM‐Scl antibody. Objectives The aims of the present study were: (i) to assess clinical features and long‐term outcome, including organ complications, functional course and mortality rate, in patients with isolated PM/DM with anti‐PM‐Scl antibody; and (ii) to evaluate prevalence, characteristics and long‐term outcome of interstitial lung disease (ILD) in patients with isolated PM/DM with anti‐PM‐Scl antibody. Methods The medical records of 20 consecutive patients with isolated PM/DM with anti‐PM‐Scl antibody were reviewed. Results Two patients (10%) achieved remission of PM/DM, whereas 14 (70%) improved and four (20%) had a worsened clinical status. Short‐term recurrences (during tapering of therapy) occurred in nine patients and long‐term recurrences (after discontinuation of therapy) in three patients. Moreover, patients with PM/DM with anti‐PM‐Scl antibody exhibited severe complications, as follows: oesophageal involvement (n = 4) requiring enteral feeding in three cases, ventilatory insufficiency (n = 3) requiring mechanical ventilation in two cases; three other patients had cancer. Interestingly, patients with PM/DM with anti‐PM‐Scl antibody often presented symptoms that are usually found in antisynthetase syndrome, i.e. hyperkeratotic rhagadiform hand symptoms (n = 2; 10%), Raynaud’s phenomenon (n = 8; 40%), arthralgia/arthritis (n = 7; 35%) and ILD (n = 12; 60%). In our cohort, the associated ILD often required combined therapy of steroids and immunosuppressive agents. Conclusions Our series suggests that the presence of anti‐PM‐Scl antibody is not a good prognostic factor in patients with PM/DM, as there appears to be an association with lung and oesophageal involvement; in addition, anti‐PM‐Scl antibody may coexist with malignancy in patients with PM/DM. Furthermore, anti‐PM‐Scl antibody‐positive patients with PM/DM often exhibit ‘mechanic’s hands’, Raynaud’s phenomenon and joint involvement. Our latter findings raise the possibility that the immunogenetic background influences the autoantibody status of these patients; HLA‐DR3 has, in fact, been found in association with antisynthetase syndrome antibodies and with anti‐PM‐Scl antibodies.     

伴恶性肿瘤皮肌炎的研究进展

皮肌炎／多发性肌炎的恶性肿瘤的伴发率约为2．5％～29．0％，中老年皮肌炎患者更易伴发恶性肿瘤，多见于40～69岁，多数皮肌炎发病后2年内发生肿瘤，可伴各种肿瘤，以鼻咽癌最常见，皮肤血管炎表现者可能与肿瘤有关，其发生机制涉及免疫学、分子生物学改变及其他等，皮肌炎症状、体征与肿瘤消长相平行，伴恶性肿瘤者疗效和预后均差。     

青少年与成人皮肌炎/多发性肌炎的比较分析

目的：探讨青少年皮肌炎/多发性肌炎（JDM/PM）的临床表现和预后特点。方法：比较分析25例JDM/PM与143例成人皮肌炎/多发性肌炎（DM/PM）的临床表现、辅助检查结果及预后情况。结果：与成人DM/PM相比,JDM/PM男多于女;初发症状中肌痛和体重下降较少见,临床表现中光敏较多见,较少出现肌痛、咽喉部肌群受累、消瘦和肺、胸膜受累;未见并发恶性肿瘤和死亡;血、尿常规,免疫学,血清酶,肌电图及肌活检结果等则与成人无统计学差异。结论：JDM/PM具有一定的临床表现特征,肺、胸膜较少累及,预后较好。     

Predicting factors of malignancy in dermatomyositis and polymyositis: a case-control study

BACKGROUND: An association between dermatomyositis (DM)/polymyositis (PM) and malignancies has been widely reported in the literature. The validity of extensive evaluation for malignancies in those patients has also been questioned for decades. Only limited papers regarding the signs of malignancy and the prognostic factors in DM/PM have been reported. OBJECTIVES: To define the potential risk factors of concomitant neoplastic diseases in patients diagnosed as having DM/PM. METHODS: From 1 April 1983 to 30 June 1999, 147 patients were diagnosed as having probable or definite DM/PM at the Veterans General Hospital, Taichung, Taiwan. We excluded four patients who had preceding neoplastic diseases diagnosed before DM/PM, then retrospectively reviewed the data of the remaining 143 patients and subgrouped the cases as four main types: primary idiopathic DM, primary idiopathic PM, juvenile DM/PM and amyopathic DM (ADM). We next performed univariate analysis using logistic regression to evaluate the possible predictive factors for malignancies, such as mean age at onset, gender, manifestations at onset, association with other connective tissue diseases, initial skin presentations, complications and laboratory data. Then we chose the significant factors for multivariate analysis by logistic regression, to determine the independent risk factors of malignancies in DM/PM patients. RESULTS: Among the 143 patients, DM was the most common type (64%), followed by ADM (14%), juvenile DM/PM (13%) and PM (10%). The mean age at onset overall was 42.4 years. Other connective tissue diseases were present in 22% of all patients, especially PM (50%) and juvenile DM/PM patients (28%). Internal malignancies were present in 13% of patients, and most were associated with DM. Nasopharyngeal carcinomas (NPCs) were the most common tumours. Patients with primary idiopathic DM, with an older age at onset, higher serum creatine phosphokinase levels and male gender, had more chance of developing concomitant malignancies. Those associated with complications, especially interstitial lung diseases, had a lower risk of associated neoplasia. In multivariate analysis, an older age at onset (odds ratio 9.10) and male gender (odds ratio 4.06) were associated with greater risk of developing malignancies. CONCLUSIONS: The two independent predictive factors for malignancy (P < 0.05) in patients with DM/PM were an older age at onset (> 45 years) and male gender. The primary idiopathic DM group was shown to have higher risk of developing internal malignancies, especially NPC. However, this was not identified as an independent predictive factor for concomitant neoplastic diseases in multivariate analysis. In addition, patients who had the complication of interstitial lung disease had a significantly lower frequency of malignancies (P < 0.001).     

Detection of the levels of neuropeptides, ACTH and cortisol in the blood of patients with polymyositis/dermatomyositis and their significance

Recent research has shown that the neuroendocrine system can regulate the function of the immune system and that ACTH and cortisol play important roles in maintaining the immune homeostasis. Polymyositis and dermatomyositis (PM/DM) are autoimmune diseases with unclear pathogeneses closely related with immune disorders, so we detected the levels of neuropeptide Y (NPY), beta-endorphin (beta-EP), calcitonin gene-related peptide (CGRP), adrenocoricotropic hormone (ACTH), and cortisol in blood of patients with PM/DM to investigate the relationship between these indices and the pathogenesis of PM/DM. The detection of NPY, beta-EP, CGRP, and ACTH concentrations in plasma and cortisol in serum of 28 cases of PM/DM was carried out using radioimmunoassay methods, and the results were compared with those of 20 normal controls. The levels of NPY in the plasma of PM/DM was significantly higher than those of the controls, while beta-EP, CGRP and ACTH were significantly lower than those of the controls, and cortisol was not significantly different before treatment. Linear correlation analysis indicated that NPY was significantly positively correlated with CPK, and beta-EP and CGRP were significantly negatively correlated with CPK. There were no significant correlations among cortisol ACTH, and CPK and no significant correlations between NPY, beta-EP, CGRP, ACTH, cortisol and age or duration of disease before treatment. After treatment for three months, NPY, beta-EP and CGRP tended to become normal and no longer significantly different from the control values. However, ACTH fell further and was significantly lower than the level before treatment. Therefore the increase in NPY and the decreases in beta-EP, CGRP, and ACTH in the plasma of PM/DM patients may be related to the pathogenesis of PM/DM.     

抗Jo-1抗体与皮肌炎和多发性肌炎临床关系的探讨

目的探讨抗Jo-1抗体与皮肌炎和多发性肌炎（PM／DM）的临床关系。方法分析16例抗Jo-1抗体阳性的多发性肌炎／皮肌炎（PM／DM）的临床特征,并与81例阴性组进行比较。结果本组中抗Jo-1抗体阳性占16．5％,抗Jo-1抗体阳性组出现肺间质病变（ILD）11例（68．75％）、肺部感染15例（93．75％）、和多关节炎／关节痛8例（50．00％）、肌肉活检异常3例（18．75％）、肌力异常4例（25．00％）、RF阳性5例（55．50％）、技工手4例（25．00％）、发热14例（87．50％）、皮肤硬肿5例（31．25％）与抗Jo-1抗体阴性组比较,差异显著（P〈0．05）。结论在多发性肌炎／皮肌炎（PM／DM）中,抗Jo—1抗体阳性者有明显的肺间质病变、肺部感染、多关节炎、RF阳性、技工手、发热、皮肤硬肿等临床特征,提高对Jo-1抗体综合征的认识,早期诊断,早期治疗,提高生存率。     

皮肌炎/多发性肌炎患者白介素4、干扰素γ、转化生长因子β1的检测

目的 探讨皮肌炎(DM)/多发性肌炎(PM)的Th1/Th2/Th3细胞功能性极化。方法 采用酶联免疫吸附试验(ELISA)测定23例DM/PM患者(DM18例,PM5例)和17例健康人的外周血单一核细胞(PBMC)培养上清液中白介素4(IL-4)、干扰素γ(IFN-γ)、转化生长因子(TGF)-β1的含量。结果 DM/PM患者的IFN-γ水平低于健康对照组(P<0.02),IL-4和TGF-β1分别高于健康对照组(P值均<0.05),IFN-γ/IL-4比值小于对照组(P<0.02)。在肺X线片或CT片上,DM10例、PM1例示肺纹理增多、增粗或纤维化,其TGF-β1分别高于无肺纹理增多、增粗或纤维化的患者(DM6例,PM2例)(P>0.05)和健康对照组(P<0.05)。结论 DM/PM患者的Th1/Th2/Th3型细胞因子网络出现异常,IL-4和TGF-β增高,IFN-γ下降。     

Polymyositis and dermatomyositis: Disease spectrum and classification

Muscle inflammation and weakness are the key features of idiopathic inflammatory myopathies (IIMs). In addition IIMs are frequently associated with cutaneous and pulmonary involvement. In clinical practice the three common inflammatory myopathies we come across are polymyositis (PM), dermatomyositis (DM) and inclusion body myositis (IBM). The Bohan and Peter criteria combine clinical, laboratory, and pathologic features to define PM and DM. They did not recognize inclusion body myositis (IBM) or other inflammatory myopathies, such as granulomatous and eosinophilic myositis. Thus the disease spectrum is wide and IIMs are a heterogeneous group of autoimmune disorders. To address these issues in this article we have discussed the currently developing newer classifications of IIMs.     

Evaluation and Management of Polymyositis

Polymyositis (PM) is one of the inflammatory myopathies, disorders characterized pathologically by the presence of inflammatory infiltrates in striated muscle. The principal clinical manifestation of PM is proximal muscle weakness. The cause of PM is unknown, but current evidence suggests that it is an autoimmune disorder. PM can affect people of any age, but most commonly presents between the ages of 50 to 70. PM is rarely seen in people younger than 18 years of age, and is twice as common among females than males. PM is more common in blacks than in whites. The overall prevalence of PM is 1 per 100,000. Muscle weakness may develop suddenly or more insidiously over a period of weeks to months. The classic symptom of PM is proximal weakness, which may manifest as difficulty holding the arms over the head, climbing stairs, or rising from a chair. Weakness of the striated muscle of the upper esophagus may result in dysphagia, dysphonia, and aspiration. The chest wall muscles may be affected, leading to ventilatory compromises. Involvement of cardiac muscle may lead to arrhythmias and congestive heart failure. Dermatomyositis (DM) is closely related to PM, and both are distinguished primarily by the occurrence of characteristic skin abnormalities in the former. PM and DM may be associated with a variety of malignancies. PM may also occur as part of the spectrum of other rheumatic diseases like systemic lupus erythematosus and mixed connective tissue disease. Moreover, inflammatory myopathy may be caused by some drugs (procainamide, D-penicillamine), and viruses, most notably the retroviruses. Corticosteroids and immunosuppressive agents are the mainstays of therapy for PM. The principal goals of therapy are to improve strength and improve physical functioning. Many patients require treatment for several years. The 5-year survival rate for treated patients is in the order of 95%. Up to one-third of PM patients may be left with some degree of residual muscle weakness.