Yamanouchi J, Yokota E, Yamauchi Y, Matsumoto I
Department of Internal Medicine, Matsuyama Red Cross Hospital.
Ryumachi. 1998 Aug;38(4):595-9.
We report a rate case of autoimmune cholangiopathy (AIC) and autoimmune hemolytic anemia (AIHA) in a patient with Sjögren's syndrome. A 59-year-old woman was admitted to Matsuyama Red Cross Hospital in September 1996 because of worsening liver dysfunction. She had suffered from keratoconjunctivitis sicca and xerostomia and had been diagnosed as having Sjögren's syndrome in February 1994, based on histological examination of the minor salivary gland and sialography, and positivity for SS-A and SS-B antibody. Liver dysfunction had first become evident in September 1995. Histological examination of a liver biopsy specimen obtained by laparoscopy showed that the structure of the hepatic lobules was mostly preserved, whereas most of the biliary ducts were transformed, being consistent with AIC. On admission, the patient was given 40 mg of prednisolone. Although the serum transaminase level decreased, jaundice persisted and hemolytic anemia developed. Further administration of 60 mg of prednisolone and plasmapheresis ameliorated the hemolytic anemia and cured the jaundice. We consider that an increased immunological response caused by the worsening AIC might have played a role in the development of AIHA in the present case.
我们报告了1例干燥综合征患者并发自身免疫性胆管病（AIC）和自身免疫性溶血性贫血（AIHA）的病例。一名59岁女性因肝功能恶化于1996年9月入住松山红十字医院。她患有干燥性角结膜炎和口干症，1994年2月根据小唾液腺组织学检查、唾液造影以及SS - A和SS - B抗体阳性被诊断为干燥综合征。肝功能障碍于1995年9月首次出现。通过腹腔镜检查获取的肝脏活检标本的组织学检查显示，肝小叶结构大多保存，但大多数胆管发生了改变，符合AIC表现。入院时，给予患者40mg泼尼松龙。尽管血清转氨酶水平下降，但黄疸持续存在且出现了溶血性贫血。进一步给予60mg泼尼松龙并进行血浆置换后，溶血性贫血得到改善，黄疸消退。我们认为，本病例中AIC病情加重导致的免疫反应增强可能在AIHA的发生中起了作用。
