Background/aims: Primary biliary cirrhosis is an autoimmune liver disease which is characterized by the presence of autoantibodies directed against mitochondrial components which belong to the pyruvate dehydrogenase enzyme complex. Apart from antibodies against mitochondrial components, primary biliary cirrhosis patients often show antibodies against nuclear components, of which anti-Sp100 and anti-gp210 are considered to be disease specific. We investigated the incidence and course of antibodies against nuclear components in primary biliary cirrhosis patients before and after liver transplantation.
Methods: Sera from 42 primary biliary cirrhosis patients were studied using indirect immunofluorescence to detect antibodies against mitochondrial components and antibodies against nuclear components, ELISA to detect anti-Sp100, and immunoblot analysis to detect anti-gp210 and antibodies against nuclear components subtypes.
Results: Ninety-three percent of primary biliary cirrhosis patients in our study were antimitochondrial antibody positive. Forty-three percent of the patients were antinuclear antibody positive. Of these, 35% had antibodies against Sp100 and 36% were positive for anti-gp210. After transplantation, antimitochondrial antibody titers as well as antinuclear antibody titers decreased in all patients. Autoantibodies in low titer persisted for up to 13 years. The pattern of nuclear autoantigens recognized by patient sera was unchanged after liver transplantation. However, the antinuclear antibody pattern was very different between the individual patients. Anti-Sp100 and anti-gp210 were not detected in sera of patients with autoimmune hepatitis, hepatitis C infection, inflammatory bowel disease, connective tissue diseases, or primary sclerosing cholangitis. The serum alkaline phosphatase level was not different in antinuclear antibody negative or positive patients before or after transplantation.
Conclusions: We conclude that the persistence of antibodies against mitochondrial components, and anti-Sp100 and anti-gp210 in primary biliary cirrhosis patients after liver transplantation is disease specific, but that this does not reflect recurrent disease activity in the graft.