Horigome H, Nomura T, Saso K, Joh T, Ohara H, Akita S, Sobue S, Mizuno Y, Kato Y, Itoh M
First Department of Internal Medicine, Nagoya City University Medical School, Japan.
Hepatogastroenterology. 2000 Jan-Feb;47(31):125-7.
We present a case that suggests a relationship between primary biliary cirrhosis and myasthenia gravis. A 43-year-old Japanese woman was admitted to the Nagoya City University Medical School, First Department of Internal Medicine with abnormal liver function in August 1991. She had had ptosis of the right eye since 1990. She had not been treated for liver disease. Ptosis of the right eye and hepatomegaly were present. Serum laboratory examinations revealed elevated biliary enzymes and IgM levels; tests were positive for antimitochondrial antibody and antiacetylcholine antibody. Liver histology revealed chronic non-suppurative destructive cholangitis and led to a diagnosis of primary biliary cirrhosis. The tensilon test was positive. Electromyography with repetitive motor nerve stimulation revealed a neuromuscular junction defect; i.e., the primary characteristic of myasthenia gravis. The patient was diagnosed with myasthenia gravis. Although the development of myasthenia gravis has previously been reported in patients with primary biliary cirrhosis during D-penicillamine administration, this is a very rare case of the coexistence of both diseases before such treatment.
我们报告一例提示原发性胆汁性肝硬化与重症肌无力之间存在关联的病例。一名43岁的日本女性于1991年8月因肝功能异常入住名古屋市立大学医学院内科第一病房。自1990年起，她就出现右眼上睑下垂。此前未针对肝脏疾病进行过治疗。查体发现右眼上睑下垂和肝肿大。血清实验室检查显示胆汁酶和IgM水平升高；抗线粒体抗体和抗乙酰胆碱抗体检测呈阳性。肝脏组织学检查显示慢性非化脓性破坏性胆管炎，诊断为原发性胆汁性肝硬化。腾喜龙试验呈阳性。重复运动神经刺激的肌电图显示神经肌肉接头缺陷，即重症肌无力的主要特征。该患者被诊断为重症肌无力。虽然此前有报道称原发性胆汁性肝硬化患者在服用青霉胺期间会发生重症肌无力，但这是一例在未进行此类治疗之前两种疾病共存的非常罕见的病例。
