医学
混合性结缔组织病
周围神经病变
尸检
肺动脉高压
肺功能测试
弱点
神经肌肉疾病
肌肉无力
病理
血浆置换术
多发性肌炎
心脏病学
外科
内科学
疾病
抗体
免疫学
糖尿病
内分泌学
作者
Martyn Jb,Wong Mj,Huang Sh
出处
期刊:PubMed
日期:1988-04-01
卷期号:15 (4): 703-5
被引量:5
摘要
We report a 34-year-old woman with mixed connective tissue disease (MCTD) who developed severe pulmonary and neuromuscular complications. At presentation, pulmonary function tests and pulmonary mechanics were suggestive of pulmonary vascular disease, and she subsequently developed clinical signs of pulmonary hypertension. These noninvasive tests may be useful in the timing of more invasive hemodynamic studies. She initially had myasthenia gravis and then developed polymyositis, profound peripheral neuropathy, and ventilatory muscle failure. She died despite aggressive immunosuppressive therapy and plasmapheresis. Autopsy showed spinal cord changes secondary to a peripheral neuropathy and signs of neurogenic atrophy confined to the ventilatory muscles. Peripheral neuropathy may be an important cause of ventilatory muscle weakness that can be found in MCTD and systemic lupus.
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