3 PATIENTS WITH AUTOIMMUNE CHOLANGIOPATHY TREATED WITH PREDNISOLONE

We describe three patients with autoimmune cholangiopathy, i.e., anti- mitochondrial antibody negative and anti-nuclear antibody-positive pri mary biliary cirrhosis, who were treated with prednisolone. Serum anti -mitochondrial antibody and anti-pyruvate dehydrogenase-E2 component a ntibody were determined by immunofluorescence of frozen sections and e nzyme-linked immunosorbent assay, respectively. Immunoblotting using m itochondria prepared from rat liver was performed to analyze anti-mito chondrial antibody in detail. Serum from one patient reacted with a 48 -kilodalton protein, but sera from the other two patients failed to re act with the mitochondrial proteins. There was a marked improvement in liver function test results after prednisolone treatment. Before trea tment, liver biopsy in all three patients showed histological features of primary biliary cirrhosis with hepatocellular necrosis. Repeat bio psy during treatment showed marked amelioration of hepatocellular dama ge in all three patients, although bile duct involvement persisted in two patients. These findings suggest that prednisolone is an effective treatment for hepatocellular damage in patients with autoimmune chola ngiopathy, but has little impact on the bile duct involvement.