We followed a case of CAH-PBC mixed type from the onset to the death for eight years. A 40-year-old woman was found to have an abnormal liver function at her age of 32. Two years later, she complained of itching during her second pregnancy and had jaundice after delivery. The serum titers of anti-nuclear antibody, anti-mitochondrial antibody, IgG and IgM were elevated. The liver biopsy revealed destruction and necrosis of intrahepatic bile ducts accompanied with chronic active hepatitis. She was diagnosed as having CAH-PBC mixed type and prednisolone was administered. As her clinical feature improved, prednisolone was discontinued, and UDCA, colestyramine and then rifampicin were administered but with little effect. Subsequently, ciclosporin was administered with some effects. However, after it was discontinued, her disease activity progressed and she died at 40-year-old. Immunologically, she had anti-M4 antibody and anti-PDC antibody. Corticosteroid was effective at the early stage but not at the end stage. Ciclosporin improved the biochemical and immunological data. UDCA was not effective. Rifampicin and colestyramine improved the complaint of itching temporally.
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