Development of autoimmune hepatitis following liver transplantation for primary biliary cirrhosis

Two patients undergoing liver transplantation for classical end-stage prima ry biliary cirrhosis (PBC) are described, who went on to develop ne novo au toimmune hepatitis (AIH) in the transplanted liver. The presentation, in bo th instances, was with malaise and lethargy Markedly elevated serum transam inases were found, together with a raised serum IgG and/or globulin fractio n and histological features on liver biopsy typical of AIH. Both cases had had changes in their immunosuppressive therapy before the onset of AIH epis odes, and both rapidly responded to reinstitution of steroid therapy. The f inding, in each case, of a coincidental multiple HLA class I allele match b etween the recipient and their liver donor suggests that HLA class I-restri cted mechanisms may play an important role in the pathogenesis of AIH.