title: Post-liver transplantation de novo hepatitis with overlap features.
abstract: The development of de novo autoimmune liver disease after liver transplantation (LT) has been described in both children and adults. Reported herein is a case that is best characterized as post-LT de novo hepatitis with features of autoimmune hepatitis (AIH)-primary biliary cirrhosis (PBC) overlap. A 56-year-old man underwent LT for decompensated liver disease secondary to non-alcoholic steatohepatitis. His liver function tests became markedly abnormal 8 months after LT. Sequential liver transplant biopsy findings were confusing and shared findings seen with both AIH and PBC. Although standard autoimmune serological tests were negative, a dramatic biochemical response was observed to a regimen consisting of prednisone, mycophenolate mofetil, and ursodeoxycholic acid added to maintainance tacrolimus. The donor was histocompatibility leukocyte antigen, DR4, positive, a haplotype associated with the development of AIH-PBC overlap syndrome. In conclusion the authors believe that this may be a case of post-LT de novo overlap syndrome of AIH-PBC, a novel 'autoimmune-type' response.
authors.affiliation: Division of Hepatology and Liver Transplantation, Lahey Clinic Medical Center, Burlington, Massachusetts, USA.
authors.forename: Andrew P
authors.initials: AP
authors.lastname: Keaveny
authors.forename: Fredric D
authors.initials: FD
authors.lastname: Gordon
authors.forename: Urmila
authors.initials: U
authors.lastname: Khettry
mesh_terms: D002756:Cholagogues and Choleretics / Q000627:therapeutic use; D004359:Drug Therapy, Combination; D019693:Hepatitis, Autoimmune / Q000188:drug therapy / Q000209:etiology* / Q000473:pathology; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008105:Liver Cirrhosis, Biliary / Q000188:drug therapy / Q000209:etiology* / Q000473:pathology; D008111:Liver Function Tests; D016031:Liver Transplantation; D008297:Male; D008875:Middle Aged; D009173:Mycophenolic Acid / Q000031:analogs & derivatives / Q000627:therapeutic use; D011183:Postoperative Complications / Q000209:etiology*; D011241:Prednisone / Q000627:therapeutic use; D016896:Treatment Outcome; D014580:Ursodeoxycholic Acid / Q000627:therapeutic use
chemical_list: D002756:Cholagogues and Choleretics; D007166:Immunosuppressive Agents; D014580:Ursodeoxycholic Acid; D009173:Mycophenolic Acid; D011241:Prednisone