...
  • 主题
高级搜索  >
历史搜索 清空历史
首页> 外文期刊>Journal of Investigative Medicine High Impact Case Reports >Necrotizing Autoimmune Myopathy: A Rare Variant of Idiopathic Inflammatory Myopathies
【24h】

Necrotizing Autoimmune Myopathy: A Rare Variant of Idiopathic Inflammatory Myopathies

机译：坏死性自身免疫性肌病：特发性炎症性肌病的罕见变体

获取原文
           
页面导航
  • 摘要
  • 著录项
  • 相似文献
  • 相关主题

摘要

Idiopathic inflammatory myopathies are an unusual group of myopathies with annual incidence of 1 in 100?000 people in the United States. Necrotizing autoimmune myopathy comprises only 16% of this group. It usually presents with severe proximal weakness, lower extremity weakness, and severe fatigue while very rarely does it present with dysphagia and respiratory muscle weakness. Statin use, cancer, and connective tissue disorder are the usual associated risk factors. Anti-signal recognition particle and 3-hydroxy-3-methylglutaryl-coenzyme A reductase are the 2 most common autoantibodies associated with necrotizing autoimmune myopathy. In this article, we present a very rare case of a 66-year-old male who presented with shortness of breath and dysphagia requiring intubation and ventilator support. Creatine kinase was 23?000, myoglobin was 7000, and ANA was positive. All other autoimmune and infectious workup including Lyme disease was unremarkable. Muscle biopsy turned out remarkable for necrotizing myopathy. No evidence of statin use, active malignancy, or connective tissue disease was found. He was treated with high-dose corticosteroids and a short course of intravenous immunoglobulin with very mild improvement in symptoms. Anti-signal recognition particle and 3-hydroxy-3-methylglutaryl-coenzyme A reductase could not be performed as the patient refused to pursue further medical testing. This is a very rare case of idiopathic inflammatory myopathy presenting with bulbar and respiratory muscle weakness requiring ventilator support.
机译：特发性炎症性肌病是一种罕见的肌病，在美国每年有100 000人中有1人发病。坏死性自身免疫性肌病仅占该组的16％。它通常表现为严重的近端无力，下肢无力和严重的疲劳，而很少出现吞咽困难和呼吸肌无力。服用他汀类药物，癌症和结缔组织疾病是常见的相关危险因素。抗信号识别颗粒和3-羟基-3-甲基戊二酰辅酶A还原酶是与坏死性自身免疫性肌病相关的2种最常见的自身抗体。在本文中，我们介绍了一个非常罕见的案例，即66岁的男性出现呼吸急促和吞咽困难，需要插管和呼吸机支持。肌酸激酶为23？000，肌红蛋白为7000，ANA为阳性。其他所有自身免疫和感染检查，包括莱姆病，均无异常。肌肉活检证明对坏死性肌病具有显着意义。没有发现使用他汀类药物，活动性恶性肿瘤或结缔组织疾病的证据。他接受了大剂量的皮质类固醇激素治疗和短暂的静脉注射免疫球蛋白治疗，症状得到了非常轻微的改善。由于患者拒绝进行进一步的医学检查，因此无法进行抗信号识别颗粒和3-羟基-3-甲基戊二酰辅酶A还原酶的检测。这是非常罕见的特发性炎症性肌病，伴有延髓和呼吸肌无力，需要呼吸机的支持。

著录项

相似文献

  • 外文文献
  • 中文文献
  • 专利
获取原文

客服邮箱：kefu@zhangqiaokeyan.com

京公网安备：11010802029741号 ICP备案号：京ICP备15016152号-6 六维联合信息科技 (北京) 有限公司©版权所有

  • 客服微信

  • 服务号