Antioxidant status of serum bilirubin and uric acid in patients with polymyositis and dermatomyositis

Objective: Oxidative stress and variations in antioxidant status are implicated in the pathogenesis of inflammatory and autoimmune diseases. Polymyositis and dermatomyositis (PM/DM) are autoimmune diseases with inflammatory cells infiltrating into skeletal muscles, and the antioxidant status is still controversial. The aim of our study was to investigate the correlation between PM/DM and the antioxidant status of serum bilirubin (Tbil, Dbil and Ibil) and uric acid (UA). Materials and Methods: We measured serum concentrations of bilirubin (Tbil, Dbil and Ibil) and uric acid in 384 individuals, including 110 PM/DM patients and 274 healthy controls. Results: We found that PM/DM patients had significantly lower serum concentrations of bilirubin (Tbil and Ibil) and uric acid than healthy controls, whether male or female. Also, after separately adjusting the covariances of age and gender, Tbil, Dbil, Ibil and UA were all relevant factors for PM/DM. Moreover, there were no significant differences in serum antioxidant molecule levels between PM and DM subgroups. Conclusion: Our study demonstrated the low serum levels of bilirubin and uric acid in patients with PM/DM. This suggested low antioxidant status in PM/DM patients with excessive oxidative stress.     

HLA-DQB1等位基因与皮肌炎/多发性肌炎相关性研究

目的　探讨 HL A- DQB1等位基因与皮肌炎 /多发性肌炎 (dermatomyositis/polymyositis,DM/PM)的相关性。方法　采用聚合酶链反应 -序列特异性引物技术 ,检测了 DM/PM患者的 HL A- DQB1等位基因。结果　与 16 0名正常对照比较 ,在 5 2例 DM/PM患者中 HL A- DQB1* 0 4 0 1等位基因频率明显增高 ,且差异有显著性 (RR=3.5 6 ,P=1.79× 10 - 3,Pc<0 .0 5 ) ;HL A- DQB1* 0 30 3的检出频率在 DM/PM患者组中有降低倾向 ,但两组差异无显著性。结论　DM/PM与 HL A- DQB1* 0 4 0 1基因有显著性相关 ,为揭示 DM/PM的发病中免疫遗传学机理所起的作用提供了重要线索和依据。     

Treatment of inflammatory myopathies

The treatment of the immune-mediated inflammatory myopathies remains largely empirical. Corticosteroids are usually effective in polymyositis and dermatomyositis but may need to be combined with methotrexate or azathioprine in some patients. Intravenous immunoglobulin (IVIg) is effective as add-on therapy in some patients not adequately controlled with steroids or immunosuppressive agents, but further controlled trials of IVIg are necessary to define the indications and optimal dose regimens. Cyclophosphamide, cyclosporin, or chlorambucil may be effective in patients with refractory polymyositis or dermatomyositis. Low-dose whole body or lymphoid irradiation is a last option in severely disabled patients resistant to all other treatments. As a small proportion of patients with inclusion body myositis respond to corticosteroid or immunosuppressive therapy, a 3–6-month trial of such therapy is justified in this condition. More specific immunotherapy for these disorders awaits identification of the target antigens and further clarification of the immunopathogenetic mechanisms. © 1997 John Wiley & Sons, Inc. Muscle Nerve 20:651–664, 1997.     

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。     

Cytokines, chemokines, and cell adhesion molecules in inflammatory myopathies

The inflammatory myopathies include dermatomyositis (DM), polymyositis (PM), and sporadic inclusion-body myositis (s-IBM). In DM, the main immune effector response appears to be humoral and directed against the microvasculature, whereas in both PM and s-IBM, cytotoxic CD8+ T cells and macrophages invade and eventually destroy nonnecrotic muscle fibers expressing major histocompatibility complex class I. The need for more specific and safer therapies in inflammatory myopathies has prompted researchers to better decipher the molecular events associated with inflammation and muscle fiber loss in these diseases. The complex specific migration of leukocyte subsets to target tissues requires a coordinated series of events, namely activation of leukocytes, adhesion to the vascular endothelium, and migration. Cell adhesion molecules (CAM) and chemokines play a major role in this multistep process. In addition, cytokines by stimulating CAM expression and orchestrating T-cell differentiation also influence the immune response. This review focuses on recent advances in defining the molecular events involved in leukocyte trafficking in inflammatory myopathies. Specific topics include a concise summary of clinical features, pathological findings and immunopathology observed in inflammatory myopathies, background information about cytokines, chemokines and cell adhesion molecules, and the expression of these molecules in inflammatory myopathies.     

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。     

A review of inflammatory idiopathic myopathy focusing on polymyositis

Inflammatory idiopathic myopathies are a group of autoimmune diseases affecting predominantly the proximal skeletal muscles, with raised muscle enzymes, with or without skin involvement and extramuscular organ involvement. Autoantibodies help to characterize patients into different clinical phenotypes. Successful treatment necessitates controlling inflammation early with corticosteroids and invariably requires additional immunosuppressive therapy. This review focuses on the aetiology, pathogenesis, clinical presentation, investigations and management of patients presenting with inflammatory idiopathic myopathies, predominantly focusing on polymyositis and antisynthetase syndrome.     

Association of anti-aminoacyl-transfer RNA synthetase antibody and anti-melanoma differentiation-associated gene 5 antibody with the therapeutic response of polymyositis/dermatomyositis-associated interstitial lung disease

Background

We attempted to clarify whether the presence of anti-aminoacyl-transfer RNA synthetase antibody (anti-ARS Ab) or anti-melanoma differentiation-associated gene 5 antibody (anti-MDA5 Ab) is associated with the therapeutic response of polymyositis/dermatomyositis-associated interstitial lung disease (PM/DM-ILD).