Primary sclerosing cholangitis
ICD-10 K83.0 · ICD-11 DB96.2

Treatment of PSC with Decompensated Cirrhosis or Hepatocellular Carcinoma

This protocol addresses primary sclerosing cholangitis in patients who have reached a stage where standard endoscopic and pharmacological measures are insufficient, or where structural disease has advanced to a critical threshold. The following clinical presentations define the population:

  • Decompensated cirrhosis
  • Hepatocellular carcinoma
  • Recurrent bacterial cholangitis
  • Severe pruritus or jaundice persisting despite endoscopic and pharmacological therapy
  • High-grade biliary dysplasia confirmed by cytology or ductal histology
Treatment Approach

Management in this setting involves a definitive surgical intervention targeting the diseased liver, with the specific biliary reconstruction technique selected according to anatomical and operative considerations. A structured post-procedure immunosuppression protocol is then initiated.

Complete regimen details — surgical approach, immunosuppression agents, sequencing, and dosing — are set out in the full structured protocol.

Instant Access to Structured Evidence-Based Regimens

References

DOI: 10.1016/j.jhep.2022.05.011

  1. Liver transplantation should be considered for people with PSC and decompensated cirrhosis or hepatocellular carcinoma according to standard guidelines.
  2. Liver transplantation should be considered for people with PSC with recurrent bacterial cholangitis and/or severe pruritus or jaundice despite endoscopic and pharmacological therapy.
  3. Liver transplantation can be considered in people with PSC and high-grade biliary dysplasia confirmed by cytology or ductal histology.
  4. Liver transplantation in PSC should be performed using a duct-to-duct anastomosis unless anatomical disease location or technical surgical factors warrant a Roux-en-Y hepaticojejunostomy.
  5. The cornerstone of immunosuppression after liver transplantation in PSC is a calcineurin inhibitor, typically tacrolimus, and whilst most experienced centres also prescribe long-term double immunosuppression by adding mycophenolate mofetil, there is no consensus and practice is variable regarding long-term, maintenance low-dose prednisolone (5 mg daily).
  6. For induction, basiliximab is now considered in most individuals with autoimmune liver disease, PSC included.
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