血清阴性坏死性自身免疫性肌病，对静脉注射免疫球蛋白反应良好。
Seronegative necrotizing autoimmune myopathy with favorable response to intravenous immunoglobulin.
作者信息
Liu Rebecca, Fenves Andrew Z, Champion Samantha N, Dau Jonathan
机构信息
Department of Medicine, Massachusetts General Hospital and Harvard Medical School, Boston, Massachusetts.
Division of Nephrology, Department of Medicine, Massachusetts General Hospital and Harvard Medical School, Boston, Massachusetts.
出版信息
Proc (Bayl Univ Med Cent). 2021 Feb 26;34(4):481-483. doi: 10.1080/08998280.2021.1885091.
We describe a 68-year-old man who presented with progressive weakness in proximal muscles of all four limbs and was found to have autoantibody-negative necrotizing autoimmune myopathy (NAM). His myopathy was refractory to corticosteroids and methotrexate, but subsequently demonstrated successful response to intravenous immunoglobulin (IVIG). The patient also received rituximab, but the timing of his recovery favored IVIG as the more important factor in terms of efficacy. Treatment guidelines for seronegative necrotizing myopathies are lacking. This case suggests a potential efficacious treatment option for the seronegative subset of NAM.
我们描述了一名68岁男性，他出现四肢近端肌肉进行性无力，被诊断为自身抗体阴性的坏死性自身免疫性肌病（NAM）。他的肌病对皮质类固醇和甲氨蝶呤治疗无效，但随后静脉注射免疫球蛋白（IVIG）显示出成功的疗效。患者还接受了利妥昔单抗治疗，但就疗效而言，他恢复的时间表明IVIG是更重要的因素。目前缺乏血清阴性坏死性肌病的治疗指南。该病例提示了NAM血清阴性亚组的一种潜在有效治疗选择。
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