Outcome of patients with ischemic-like cholangiopathy with secondary sclerosing cholangitis after liver transplantation

Scand J Gastroenterol. 2011 Apr;46(4):471-8. doi: 10.3109/00365521.2010.537683. Epub 2010 Nov 30.


Background and aims: Sclerosing cholangitis in critically ill patients (SC-CIP) with sepsis and acute respiratory distress syndrome (ARDS) is a cholestatic liver disease with a rapid progression to liver cirrhosis and hepatic failure. Data on outcome of these patients after liver transplantation (LT) are sparse.

Patients and methods: Eleven patients (46 ± 12 years; mean labMELD-score: 27 ± 7) with SC-CIP underwent LT. Six patients had severe polytrauma with multiple bone fractures, sepsis and ARDS. Five non-traumatic patients acquired SC-CIP during long-term intensive-care-unit stays due to sepsis and ARDS. Time to diagnosis, the microbiologic results and the survival rates after LT were evaluated.

Results: SC-CIP was diagnosed by endoscopic retrograde cholangiopancreatography (ERCP) within 3 ± 1 months after manifestation of cholestasis and histologically confirmed in explanted livers. The predominant microorganisms isolated in bile were: Enterococcus and Candida albicans. Mean follow-up after LT was 28 ± 20 months. One female patient (non-traumatic) died due to sepsis 26 days after LT. All other patients left the hospital alive, but two (non-traumatic) patients died from sepsis, and one (traumatic) patient died in a hemorrhagic shock, thereafter. Seven of 11 patients (5 with polytrauma) are still alive and have a good quality of life. The survival of the SC-CIP patients after LT was comparable with that of patients transplanted due to alcoholic liver cirrhosis.

Conclusion: SC-CIP develops rapidly within several months. Enterococcus and C. albicans were the main isolated microorganisms in the bile. Sepsis was the main cause of death after LT. Overall, SC-CIP is a good indication for LT in selected patients.

MeSH terms

  • Adult
  • Aged
  • Bile / microbiology
  • Candida albicans
  • Cholangitis, Sclerosing / complications
  • Cholangitis, Sclerosing / microbiology
  • Cholangitis, Sclerosing / mortality
  • Cholangitis, Sclerosing / surgery*
  • Critical Care
  • Critical Illness
  • Enterococcus faecalis / isolation & purification
  • Enterococcus faecium / isolation & purification
  • Female
  • Humans
  • Liver Cirrhosis, Alcoholic / mortality
  • Liver Cirrhosis, Alcoholic / surgery*
  • Liver Transplantation*
  • Male
  • Middle Aged
  • Respiratory Distress Syndrome / complications
  • Sepsis / complications
  • Survival Rate
  • Treatment Outcome