li-11-11 Idiopathic adulthood ductopenia By Dr. E. Orfei
Vanishing bile duct syndrome with jaundice and pruritus which lacks all characteristics and antibodies
associated with other vanishing bile duct conditions.
Two types of this syndrome are recognized.
Type one include patients who are asymptomatic or present only cholestasis. They have less bile duct destruction
on liver biopsy and their clinical course ranges from spontaneous improvement to development of biliary cirrhosis.
Type two include those patients presenting decompensated biliary cirrhosis and have marked destruction of intrahepatic bile ducts and require orthotopic liver transplantation which will result in complete resolution of the jaundice and pruritus. (Arch Intern Med.160:1033, 2000).