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自身免疫性坏死性抗 HGMCR 肌病引起的吞咽困难。

Dysphagia secondary to autoimmune necrotizing anti-HGMCR myopathy.

机构信息

Aparato Digestivo, Complejo Hospitalario Universitario de A Coruña, España.

Aparato Digestivo, Complejo Hospitalario Universitario de A Coruña.

出版信息

Rev Esp Enferm Dig. 2021 Oct;113(10):729-730. doi: 10.17235/reed.2020.7324/2020.

Abstract

We present the case of an 87-year-old female who presented with upper dysphagia to solids, weight loss of 5 kg and weakness in the upper limbs of a seven months duration. Gastroscopy showed a hiatal hernia and an esophagogram showed gastroesophageal reflux and contrast aspiration into the trachea. Esophageal manometry was not possible due to intolerance. Analytics showed elevated CPK (3,386 UI/l; 34-145) and positivity to anti-HMGCR antibodies. An electromyogram was performed, with more intense myopathy data in the proximal regions of the upper limbs and signs of myositis. A muscle biopsy was performed (deltoids and biceps) that showed signs of necrotizing myopathy. Treatment with oral steroids and methotrexate was started with a good subsequent evolution.

摘要

我们现呈现一例 87 岁女性病例,其主要表现为进行性固体食物吞咽困难、7 个月体重减轻 5 公斤以及上肢无力。胃镜检查发现食管裂孔疝,食管造影显示胃食管反流和对比剂吸入气管。由于不耐受,食管测压无法进行。分析显示肌酸激酶升高(3386 UI/L;34-145)和抗 HMGCR 抗体阳性。进行了肌电图检查,上肢近端区域的肌病数据更为明显,并出现肌炎迹象。进行了肌肉活检(三角肌和肱二头肌),显示出坏死性肌病的迹象。开始口服类固醇和甲氨蝶呤治疗,随后病情良好。

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