原发性胆汁性肝硬化（PBC）-CREST（钙质沉着、雷诺现象、食管功能障碍、指（趾）硬皮病和毛细血管扩张）重叠综合征合并干燥综合征和关节炎。

Primary biliary cirrhosis (PBC)-CREST (calcinosis, Raynaud's phenomenon, esophageal dysfunction, sclerodactyly and telangiectasia) overlap syndrome complicated by Sjögren's syndrome and arthritis.

作者信息

Ito M, Kojima T, Miyata M, Saka M, Kokubun M, Ohira H, Ishikawa H, Kuroda M, Sato Y, Takagi T

机构信息

Department of Internal Medicine II, Fukushima Medical College.

出版信息

Intern Med. 1995 May;34(5):451-4. doi: 10.2169/internalmedicine.34.451.

DOI:10.2169/internalmedicine.34.451

PMID:7647420

Abstract

A 36-year-old woman with primary biliary cirrhosis (PBC)-CREST (calcinosis, Raynaud's phenomenon, esophageal dysfunction, sclerodactyly and telangiectasia) overlap syndrome complicated by Sjögren's syndrome and arthritis is reported. She had suffered from Raynaud's phenomenon, sclerodactyly, morning stiffness, arthralgia and sicca symptoms since 34 years of age. She exhibited an increased level of alkaline phosphatase and hyperglobulinemia at 2.8 g/dl without any symptoms, and histological findings from the biopsy specimen of the liver were consistent with those of PBC. Her human leukocyte antigen (HLA) typing was DR8 homozygous.

摘要

报道了一名36岁患有原发性胆汁性肝硬化（PBC）-CREST（钙质沉着、雷诺现象、食管功能障碍、指端硬化和毛细血管扩张）重叠综合征并伴有干燥综合征和关节炎的女性。她自34岁起就患有雷诺现象、指端硬化、晨僵、关节痛和口眼干燥症状。她在没有任何症状的情况下碱性磷酸酶水平升高且球蛋白血症高达2.8g/dl，肝脏活检标本的组织学检查结果与PBC相符。她的人类白细胞抗原（HLA）分型为DR8纯合子。