Criteria for Diagnosis of Progressive Intrahepatic Cholestasis

Chronic cholestasis, leading to progressive liver injury with hepatic insufficiency and fibrosis on biopsy	
Normal biliary tree, both extrahepatic (excluding sclerosing cholangitis) and intrahepatic (excluding paucity)	
Modest or no elevation in GGT and cholesterol levels	
Absence of abnormalities in other organ systems	
Other disorders, such as diseases of bile acid formation, excluded