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结节病中类似硬化性胆管炎的弥漫性肝内胆管狭窄。病例报告及文献复习。

Diffuse intrahepatic biliary strictures in sarcoidosis resembling sclerosing cholangitis. Case report and review of the literature.

作者信息

Alam I, Levenson S D, Ferrell L D, Bass N M

机构信息

Department of Medicine, University of California, San Francisco 94143, USA.

出版信息

Dig Dis Sci. 1997 Jun;42(6):1295-301. doi: 10.1023/a:1018874612166.

Abstract

We report a case of sarcoidosis with severe cholestasis and cholangiographic features of sclerosing cholangitis that responded dramatically to corticosteroid therapy. Although an association between sarcoidosis and primary sclerosing cholangitis has been suggested by previous reports, features suggestive of primary sclerosing cholangitis, including inflammatory bowel disease, hepatic histology and serum neutrophil cytoplasmic antibodies, were absent in this case. Cholangiography may be useful in the evaluation of patients with cholestatic sarcoid liver disease, and intrahepatic biliary strictures should be included in the spectrum of hepatic involvement by sarcoidosis. A trial of corticosteroid therapy may be of benefit in patients with bile ductal involvement by sarcoidosis.

摘要

我们报告一例结节病患者,其伴有严重胆汁淤积及具有硬化性胆管炎的胆管造影特征,对皮质类固醇治疗反应显著。尽管既往报告提示结节病与原发性硬化性胆管炎之间存在关联,但该病例不存在提示原发性硬化性胆管炎的特征,包括炎症性肠病、肝脏组织学及血清中性粒细胞胞浆抗体。胆管造影在评估胆汁淤积性结节病肝病患者中可能有用,肝内胆管狭窄应纳入结节病肝脏受累范围。对于结节病累及胆管的患者,试用皮质类固醇治疗可能有益。

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