Open AccessDuctopenia related liver sarcoidosis
Author(s) -
Nourr-Eddine Farouj,
Jean–François Cadranel,
Ali Mofredj,
Vincent Jouannaud,
M. Lahmiri,
Pierre Le Lann,
A Cazier
Publication year - 2011
Publication title -
world journal of hepatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.913
H-Index - 55
ISSN - 1948-5182
DOI - 10.4254/wjh.v3.i6.170
Subject(s) - medicine , sarcoidosis , asymptomatic , primary biliary cirrhosis , jaundice , cholestasis , gastroenterology , pathology , primary sclerosing cholangitis , cirrhosis , liver disease , disease
Sarcoidosis is a systemic granulomatous disease which may involve many organs. In approximately 95% of patients there is liver involvement, with noncaseating hepatic granulomas occurring in 21 to 99% of patients with sarcoidosis. Liver involvement is usually asymptomatic and limited to mild to moderate abnormalities in liver biochemistry. The occurrence of jaundice in sarcoidosis is rare; extensive imaging procedures and the examination of liver biopsies permit a precise diagnostic. Ductopenia associated with sarcoidosis has been reported in less than 20 cases and can lead to biliary cirrhosis and liver- related death. We report here on a case of ductopenia-related sarcoidosis in which primary biliary cirrhosis and extrahepatic cholestasis have been carefully excluded. The patient follow up was 8 years. Although ursodesoxycholic acid appears to improve liver biochemistry it does not preclude the rapid occurrence of extensive fibrosis. A review of the literature of reported cases of ductopenia related to sarcoidosis is provided.