Autoimmune Cholangitis: A Unique Entity?

Abstract

Overlap syndromes between cholestatic chronic hepatitis and primary biliary cirrhosis are being increasingly discussed. The diagnosis depends particularly on the pattern of serum autoantibodies, whether suggestive of one condition or the other. The autoantibodies have an important diagnostic role, but their contribution in mediating bile duct and hepatocellular injury is uncertain. In this report, five patients (three women and two men) are described with hepatic histologic features resembling primary biliary cirrhosis but with negative results for serum antimitochondrial antibody (M2) tests. Serum antinuclear antibody of diffuse type is strongly positive. The serum transaminase levels are 4 to 6 times the upper limit of normal, and serum γ-glutamyl transpeptidase values are substantially increased. The response to pred-nisolone therapy is partial: inflammation is reduced, but the serum γ-glutamyl transpeptidase level remains high and bile duct lesions persist. This condition, which is an overlap between primary biliary cirrhosis and hepatitis, has been termed autoimmune cholangitis. Treatment with ursodeoxycholic acid is recommended, and prednisolone therapy may be considered even though beneficial results have not been impressive.