Recurrence of autoimmune disease, primary sclerosing cholangitis, primary biliary cirrhosis, and autoimmune hepatitis after liver transplantation

Abstract

1. Recurrence of primary sclerosing cholangitis. a Five percent of liver transplants are performed because of end-stage liver disease secondary to primary sclerosing cholangitis. b Recurrent disease affects 10% to 27% of recipients. c Diagnostic criteria of recurrence include the following: A confirmed diagnosis of primary sclerosing cholangitis before liver transplantation. A cholangiogram showing nonanastomotic biliary strictures with beading and irregularity occurring 90 days after transplantation. Liver biopsy revealing fibrous cholangitis and/or fibro-obliterative lesions of large bile ducts. d The data argue for an association between recurrent primary sclerosing cholangitis and rejection and steroid therapy. 2. Recurrence of primary biliary cirrhosis. a Overall recurrence rates can reach 50%. b The gold standard for diagnosis is histological, with bile duct destruction by granulomas indicated by a florid lesion. c Anti-mitochondrial antibody is not a reliable marker of recurrence. d Recurrence does not affect long-term patient or graft survival. 3. Recurrence of autoimmune hepatitis. a Recurrence affects approximately 25% of liver allografts during the first 5 years after liver transplantation and more than 50% after 10 years of follow-up. b Diagnostic criteria of recurrence must include a combination of biochemical changes, (elevated serum aminotransferases levels and hypergammaglobulinemia), histological features of autoimmune hepatitis, and steroid dependency. c Immunosuppressive therapy should be pursued even if liver test results are normal.