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两例伴有CREST综合征的无症状原发性胆汁性肝硬化(PBC)

[Two cases of asymptomatic primary biliary cirrhosis (PBC) accompanied with CREST syndrome].

作者信息

Takeda I, Igarashi S, Oyanagi H, Irisawa A, Mukai S, Shoji I, Sakuma H, Satho H, Sekine K, Kuroda M

机构信息

Department of Internal Medicine, Ohota Nishinouchi Hospital, Fukushima.

出版信息

Ryumachi. 1994 Aug;34(4):767-72.

PMID:7974028
Abstract

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.

摘要

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。

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