[IgG4 associated autoimmune cholangitis].

Immunoglobulin G4 associated autoimmune cholangitis (IAC) is a recently described cholangiopathy, which closely resembles primary sclerosing cholangitis (PSC) and belongs to IgG4 sclerosing disease family. Unlike PSC the disease is most commonly seen in elderly men presenting with abdominal pain and obstructing jaundice as the first symptoms. In cholangiograms, distal biliary tract changes are most frequent. IAC is seldom associated with inflammatory bowel disease, less than in 10 % of cases. Differential diagnosis between Iac and PSC is essential because both the therapy and prognosis are totally different. Definite diagnosis is based on immunohistology or concomitant presentation of other IgG4 associated sclerosing diseases in addition to elevated IgG4-serum concentration. Steroid therapy has been shown to ameliorate symptoms and leads to regression of bile duct abnormalities and normalizes the liver function tests.