Idiopathic Biliary Ductopenia in Adults without Symptoms of Liver Disease

BACKGROUND: Idiopathic adulthood ductopenia is a severe cholestatic liver disease of unknown cause characterized by loss of the interlobular bile ducts in more than 50 percent of the portal tracts. In most reported cases, cirrhosis and liver failure develop. METHODS: We studied 24 adults with abnormal results on liver-function tests but no symptoms of liver disease. All had liver biopsies that showed a lack of bile ducts in many of the portal tracts. RESULTS: The 17 women and 7 men had a mean age of 41 years (range, 27 to 57). All were asymptomatic and had high serum gamma-glutamyltransferase concentrations (mean [+/-SD], 179 +/- 84 U per liter); 75 percent also had abnormal serum alanine aminotransferase concentrations. The proportion of portal tracts that had bile ducts was 62 +/- 7 percent (range, 55 to 78 percent). Three patients had a second liver biopsy three to nine years after the first; there were no changes over time. In four of the five patients treated with 600 to 900 mg of ursodiol two to three times daily, results of liver-function tests returned to normal. CONCLUSIONS: Idiopathic biliary ductopenia, with an apparently nonprogressive clinical course, can occur in adults who have no symptoms of biliary disease.