Late graft dysfunction after liver transplantation can be induced by infections, vascular complications, biliary problems, recurrence of the original disease, lymphoproliferative disease, poor compliance leading to cellular rejection, or onset of chronic rejection. We have recently described a novel form of graft dysfunction in the absence of these causes. The characteristic features were strikingly reminiscent of classical autoimmune liver disease (AILD), including hypergammaglobulinaemia, elevated titres of serum autoantibodies, and histo-logical findings of chronic hepatitis with portal and periportal infiltrate with plasma cells and lymphocytes, perivenular cell necrosis, bridging fibrosis and collapse. Furthermore, six out of seven patients have responded to standard treatment of AILD with prednisolone (2mgkg~(-1)d~(-1)) and azathioprine (1.5-2mgkg~(-1)d~(-1)) within a median of 32 days (range 7-316). Four of the patients fulfilled the international criteria for 'definite' and three for 'probable' autoimmune hepatitis. None of the children was transplanted for AILD and all had serum levels of cyclosporine A or tacrolimus within the therapeutic range at diagnosis. Three patients had low titres of serum autoantibodies before transplantation. During the six-year observation period, the overall incidence of de-novo autoimmune hepatitis amongst children transplanted at our centre was 4%. It is noteworthy that five patients had received allografts from donors with HLA DR3/DR4 alleles, known to confer susceptibility to AILD. However, when we compared the HLA alleles of the children who developed de-novo autoimmune hepatitis with those of 32 children matched for length of post-transplant follow-up, we could not detect a statistical difference.
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