Post-liver transplantation de novo hepatitis with overlap features.
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.
Keaveny
Andrew P
AP
Division of Hepatology and Liver Transplantation, Lahey Clinic Medical Center, Burlington, Massachusetts, USA.
Gordon
Fredric D
FD
Khettry
Urmila
U
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
D002756:Cholagogues and Choleretics; D007166:Immunosuppressive Agents; D014580:Ursodeoxycholic Acid; D009173:Mycophenolic Acid; D011241:Prednisone