Idiopathic adulthood ductopenia - Case report and review of the literature

The clinical and pathological findings of idiopathic ductopenia were studie d in a 30-year-old woman who initially manifested jaundice and pruritus, Se rum biochemical tests of liver function indicated severe and progressive ch olestasis. Viral hepatitis markers and circulating autoantibodies were abse nt. The patient had a normal cholangiogram and lacked evidence of inflammat ory bowel disease. Histological examination of a liver specimen showed seve re cholestasis and absence of interlobular bile ducts. Severe jaundice and intractable pruritus developed in the patient and served as the indications for liver transplantation 4 months after initial examination, Transplantat ion resulted in prompt and complete resolution of the jaundice and pruritus . Two types of idiopathic adulthood ductopenia associated with different pr ognoses are recognized. Patients with type 1 idiopathic adulthood ductopeni a are asymptomatic or manifest symptoms of cholestatic liver disease. They tend to have less destruction of the intrahepatic bile ducts on liver biops y specimens. Their clinical course ranges from spontaneous improvement to p rogression to biliary cirrhosis. In contrast, patients with type 2 idiopath ic adulthood ductopenia generally manifest initial symptoms of decompensate d biliary cirrhosis, have extensive destruction of the intrahepatic bile du cts on liver biopsy, and frequently require orthotopic liver transplantatio n.