Post-liver transplant cholestatic disorder with biliary strictures: de novo versus recurrent primary sclerosing cholangitis.
Cholestatic allograft dysfunction following liver transplantation (LT) can result from many different underlying pathogenetic mechanisms and is a major cause of morbidity and graft loss. Although recurrence of primary sclerosing cholangitis (PSC) is a described entity following LT, the diagnosis is difficult and requires exclusion of common risk factors for stricture formation. There are no reports in the literature of de novo PSC arising in a patient who did not have that disease prior to transplantation. Reported herein is the case of a patient who underwent transplantation for end-stage cryptogenic cirrhosis and who had no underlying risk factors, but who developed late post-LT cholestatic disorder with non-anastomotic biliary strictures. The combined clinical, radiological, and pathological findings resembled those of PSC. Admittedly, it is a challenging proposition but the possibility of a de novo PSC-like syndrome in this patient is raised. A recurrence in a patient who may have had a burnt-out, PSC-like syndrome presenting as cryptogenic cirrhosis, however, cannot be entirely excluded.
Department of Transplantation, Baystate Medical Center, Springfield, MA 01107, USA. mcpartlandkenneth@hotmail.com
Kenneth Joseph
KJ
McPartland
William David
WD
Lewis
Fredric David
FD
Gordon
Elizabeth Anne
EA
Pomfret
James John
JJ
Pomposelli
Roger
R
Jenkins
Urmila
U
Khettry
D000368:Aged; D000893:Anti-Inflammatory Agents / Q000627:therapeutic use; D001653:Bile Ducts, Intrahepatic / Q000473:pathology*; D002756:Cholagogues and Choleretics / Q000627:therapeutic use; D015209:Cholangitis, Sclerosing / Q000188:drug therapy / Q000209:etiology / Q000473:pathology*; D006801:Humans; D008103:Liver Cirrhosis / Q000601:surgery; D016031:Liver Transplantation / Q000009:adverse effects*; D008297:Male; D011183:Postoperative Complications / Q000188:drug therapy / Q000209:etiology / Q000473:pathology*; D011241:Prednisone / Q000627:therapeutic use; D014580:Ursodeoxycholic Acid / Q000627:therapeutic use
D000893:Anti-Inflammatory Agents; D002756:Cholagogues and Choleretics; D014580:Ursodeoxycholic Acid; D011241:Prednisone