Cordeiro A C, Isenberg D A
Department of Rheumatology, Hospital Garcia De Orta, Avenida Torrado Da Silva, Pragal, 2801-951 Almada, Portugal.
Postgrad Med J. 2006 Jul;82(969):417-24. doi: 10.1136/pgmj.2005.038455.
Idiopathic inflammatory myopathies, notably polymyositis and dermatomyositis are comparatively uncommon diseases and few randomised, double blind placebo controlled trials have been done. Final validation of measures to assess outcome and response to treatment is awaited. Corticosteroids are an effective initial treatment, although rarely tested in randomised controlled trials. Unfortunately, not all patients respond to them and many develop undesirable side effects. There is thus a need for second line agents notably immunosuppressives or intravenous immunoglobulin. There are no defined guidelines or best treatment protocols agreed internationally and so the medical approach must be individualised, based on the severity of clinical presentation, disease duration, presence of extramuscular features, and prior therapy and contraindications to particular agents. There is still a significant percentage of non-responders (around 25%) and clinical relapses. Novel therapeutic approaches are now directed towards cytokine modulation and the use of monoclonal antibodies targeting B and T cells.
特发性炎性肌病,尤其是多发性肌炎和皮肌炎,是相对罕见的疾病,很少有随机、双盲、安慰剂对照试验。评估治疗结果和反应的措施的最终验证仍有待完成。皮质类固醇是一种有效的初始治疗方法,尽管在随机对照试验中很少进行测试。不幸的是,并非所有患者对其有反应,许多患者会出现不良副作用。因此需要二线药物,尤其是免疫抑制剂或静脉注射免疫球蛋白。目前尚无国际公认的明确指南或最佳治疗方案,因此医疗方法必须根据临床表现的严重程度、疾病持续时间、肌肉外特征的存在、先前的治疗以及特定药物的禁忌症进行个体化。仍有相当比例的患者无反应(约25%)且会出现临床复发。新型治疗方法目前正朝着细胞因子调节以及使用针对B细胞和T细胞的单克隆抗体的方向发展。