Takeda I, Igarashi S, Oyanagi H, Irisawa A, Mukai S, Shoji I, Sakuma H, Satho H, Sekine K, Kuroda M
Department of Internal Medicine, Ohota Nishinouchi Hospital, Fukushima.
Ryumachi. 1994 Aug;34(4):767-72.
Two cases of asymptomatic primary biliary cirrhosis (PBC) combining CREST syndrome. We have had encountered two primary biliary cirrhosis (PBC) patients overlapped with CREST syndrome. Case 1 was a 51-year-old female, who was suffering from Raynaud's phenomenon, esophageal dysmotility and sclerodactyly. Case 2 was a 67-year-old female, who was suffering from Raynaud's phenomenon, esophageal dysmotility, sclerodactyly and telangiectasia. They were free from itching and icterus. The histology of their biopsied liver specimen should stage I-II of Scheuer's classification. Their immunological findings showed anti-centromere antibody, (ACA) at a high titer (1 : 1280 dilution) and anti-mitochondrial antibody (AMA) at a low titer (1 : 40 dilution) positive in both. HLA DR types included DR2 and DRW8 in case 1, and did DR1 and DRW6 in case 2. Both patients are having good prognosis.
两例无症状原发性胆汁性肝硬化（PBC）合并CREST综合征。我们遇到了两例原发性胆汁性肝硬化（PBC）患者，他们同时患有CREST综合征。病例1是一名51岁女性，患有雷诺现象、食管动力障碍和指（趾）硬皮病。病例2是一名67岁女性，患有雷诺现象、食管动力障碍、指（趾）硬皮病和毛细血管扩张。她们没有瘙痒和黄疸症状。她们活检肝脏标本的组织学检查符合Scheuer分类的I-II期。她们的免疫学检查结果显示，两人均抗着丝点抗体（ACA）呈高滴度（1:1280稀释）阳性，抗线粒体抗体（AMA）呈低滴度（1:40稀释）阳性。病例1的HLA DR类型包括DR2和DRW8，病例2的HLA DR类型为DR1和DRW6。两名患者预后良好。
