APHERESIS IN THE TREATMENT OF POLYMYOSITIS/DERMATOMYOSITIS

多发性肌炎和皮肌炎是罕见的自身免疫性疾病 病因学。然而，大多数患者都有对强的松敏感的疾病， 大约20%的PM/DM患者要么对类固醇没有反应， 治疗反应不完全，或有无法忍受的不良反应。在……里面 这些个体，细胞毒性药物，如硫唑嘌呤或甲氨蝶呤 通常是给予的。对细胞毒药物的不良反应发生率很高， 然而，相当大比例的患者将保持无反应 或者不能忍受治疗。 许多轶事报道描述了PM/DM症状的改善 对血浆交换或白细胞分离术的反应。在大多数的 在这些病例中，使用类固醇和/或细胞毒剂进行治疗。 同时，分离的效果无法分开分析 与其他疗法不同。这项研究的目的是定义 采集术在治疗PM/DM中的作用 虚假控制的双盲时尚。 对大剂量强的松无反应或不耐受的患者 随机分为：(1)血浆置换，(2)白细胞分离术，或(3)假手术 采集术。每周分离3次，共4次。 几周。患者和研究人员评估对治疗的反应 对所执行的程序视而不见。没有这样做的患者 随后给予环磷酰胺1.0g/M2静脉注射 每月一次，为期6个月。42名患者进入了这项研究， 这项研究于1990年6月结束，以获得进一步的患者收益。分析了 肌力、CPK、醛缩酶、LDH、SGOT和肌肉活检的反应 在42名参与者中，目前正在进行中。

Polymyositis and dermatomyositis are uncommon diseases of autoimmune etiology. Most patients have disease responsive to prednisone, however, approximately 20% of PM/DM patients either do not respond to steroids, respond incompletely, or have intolerable adverse effects of therapy. In these individuals, cytotoxic drugs such as azathioprine or methotrexate are often given. The rate of adverse reactions to cytotoxic agents is high, however, and a significant proportion of patients will remain nonresponsive or intolerant of therapy. A number of anecdotal reports describe improvement in symptoms of PM/DM in response to either plasma exchange or leukapheresis. In the majority of these cases, therapy with steroids and/or cytotoxic agents was given simultaneously, and the effect of apheresis could not be analyzed apart from that of other therapies. The purpose of this study was to define the role of apheresis in the treatment if PM/DM in a randomized, sham-controlled, double-blind fashion. Patients non-responsive or intolerant of high-dose prednisone were randomized to either: (1) plasma exchange, (2) leukapheresis, or (3) sham apheresis. The apheresis procedures were performed 3 times weekly for 4 weeks. The patients and the investigators assessing response to therapy were kept blinded as to the procedures performed. Patients who did not improve with apheresis were subsequently given cyclophosphamide 1.0 g/M2 IV monthly for 6 months. Forty-two patients have entered the study and the study closed to further patient accrual in June, 1990. Analyses of responses in muscle strength, CPK, aldolase, LDH, SGOT, and muscle biopsy in the 42 participants are currently in progress.