多发性肌炎/皮肌炎治疗研究进展

多发性肌炎/皮肌炎(polymyositis,PM/dermatomyositis,DM)是以侵犯骨骼肌为主,可累及肌肉、肺、肾等多个脏器的自身免疫性疾病,其临床表现复杂,患者多因肺间质病变、肿瘤等并发症死亡,治疗以糖皮质激素为主,以及免疫抑制剂、生物制剂等,但不良反应大,对相关并发症的效果不理想。而干细胞移植治疗是作为当前治疗难治性PM/DM的一种手段。本文从PM/DM的治疗现状出发进行综述,以期为PM/DM临床治疗提供更多参考。     

多发性肌炎与甲状腺功能减退性肌病的鉴别诊断

目的探讨多发性肌炎与甲状腺功能减退性肌病的异同点以减少误诊。方法回顾分析4例多发性肌炎和4例甲状腺功能减退性肌病患者的临床表现、实验室检查资料及治疗转归。结果多发性肌炎与甲状腺功能减退性肌病的丙氨酸转氨酶、肌酸激酶、肌肉病理有明显统计学差异,甲状腺功能检查结果、治疗转归明显不同。结论对于肌肉无力和肌酶升高的患者需常规检测甲状腺功能,肌肉病理、诊断性甲状腺素替代治疗对鉴别多发性肌炎和甲状腺功能减退症肌病所致肌肉病变有重要意义。     

重叠综合征并发横纹肌溶解症一例

74岁女性患者,手足及口周部位皮肤变硬2个月余,伴乏力、酱油色尿1个月。血清肌酸激酶4 242 U/L,肌红蛋白1 124 ng/ml。诊断为重叠综合征并发横纹肌溶解症。经甲泼尼龙80 mg每日1次、甲氨蝶呤15 mg每周1次治疗,效果不理想,转入肾病内科行血液净化治疗。血清肌红蛋白恢复正常后,继续口服甲泼尼龙40 mg每日1次,定期复诊并缓慢减量。目前甲泼尼龙减量至16 mg/d,己持续治疗1年,血清肌酸激酶及肌红蛋白均在正常范围。     

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.     

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.     

多发性肌炎发病机制中的血管因素研究

目的　研究多发性肌炎中细胞间黏附分子 1(ICAM 1)、IL 1α在微血管的表达情况,探讨血管因素在多发性肌炎中的作用。方法　收集 10例多发性肌炎患者和 6例非炎性肌病,采用图像分析法计算CD34、ICAM 1、IL 1α阳性微血管面积与面密度,进行定量分析和比较。结果　多发性肌炎患者肌组织中微血管面密度较非炎性肌病组明显减少 (P=0 009 );微血管内皮ICAM 1、IL 1α的表达强度较对照组增强。结论　多发性肌炎患者肌组织微循环功能受损,提示血管因素可能参与了多发性肌炎的发病。