Saadeh C, Bridges W, Burwick F
Department of Internal Medicine, Texas Tech University Health Sciences Center at Amarillo 79106, USA.
South Med J. 1995 Aug;88(8):866-70. doi: 10.1097/00007611-199508000-00018.
Dermatomyositis is an uncommon idiopathic chronic inflammatory disorder. Oral corticosteroids are the treatment of choice. A few patients become resistant to steroids. We describe two patients who, after failure to respond to oral and high-dose intravenous corticosteroids, received a combination of oral cyclosporine and high-dose intravenous immune globulin, with apparent remission. The first patient was a 6-year-old girl with recurrent disease and vasculitis, despite prednisone therapy and normal muscle enzyme levels. The response was remarkable within 3 weeks of therapy. The second patient was a 30-year-old woman with progressive disease and secondary respiratory failure despite oral prednisone and methotrexate therapy. The response to treatment was optimal within 4 weeks. She received maintenance low-dose cyclosporine and a tapering dose of prednisone. The combination of high-dose immune globulin and cyclosporine can be useful and safe in the management of steroid-resistant dermatomyositis.
皮肌炎是一种罕见的特发性慢性炎症性疾病。口服糖皮质激素是首选治疗方法。少数患者会对类固醇产生耐药性。我们描述了两名患者，在对口服和大剂量静脉注射糖皮质激素治疗无反应后，接受了口服环孢素和大剂量静脉注射免疫球蛋白的联合治疗，病情明显缓解。第一名患者是一名6岁女孩，尽管接受了泼尼松治疗且肌肉酶水平正常，但仍患有复发性疾病和血管炎。治疗3周内反应显著。第二名患者是一名30岁女性，尽管接受了口服泼尼松和甲氨蝶呤治疗，但病情仍进展并出现继发性呼吸衰竭。治疗4周内反应最佳。她接受了低剂量环孢素维持治疗和逐渐减量的泼尼松治疗。大剂量免疫球蛋白和环孢素联合应用在治疗对类固醇耐药的皮肌炎方面可能有效且安全。
