Recurrence of Autoimmune Disease, Primary Sclerosing Cholangitis, Primary Biliary Cirrhosis, and Autoimmune Hepatitis After Liver Transplantation

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. Liver Transpl 15: S25-S34, 2009. (C) 2009 AASLD.