Liver transplantation in primary biliary cholangitis (primary biliary cirrhosis)
- Steven Flamm, MD
Steven Flamm, MD
- Chief, Liver Transplantation Program
- Professor of Medicine
- Feinberg School of Medicine
- Northwestern University
- Fredric D Gordon, MD
Fredric D Gordon, MD
- Associate Professor of Medicine
- Tufts University Medical School
- Raoul Poupon, MD
Raoul Poupon, MD
- Professor of Hepatology and Gastroenterology
- University Pierre et Marie Curie
- UPMC, Sorbonne University, Paris, France
- Section Editor
- Robert S Brown, Jr, MD, MPH
Robert S Brown, Jr, MD, MPH
- Section Editor — Liver Transplantation
- Vice Chair, Transitions of Care, Department of Medicine
- Interim Chief, Division of Gastroenterology and Hepatology
- Weill Cornell Medical College
- Professor of Clinical Medicine, Columbia University College of Physicians & Surgeons
Liver transplantation can be successful in treating end-stage liver disease from primary biliary cholangitis (PBC; previously referred to as primary biliary cirrhosis). The total number of transplants performed for PBC in recent years has declined slightly, possibly reflecting benefits of early treatment . Nevertheless, transplantation remains an important option in patients with progressive disease despite medical therapy. In the United States, the average age of patients undergoing transplantation for PBC is in the range of 53 to 55 years .
This topic will review issues related to patient selection for liver transplantation, the timing of transplantation, and transplantation outcomes in patients with PBC. Other issues related to the pathogenesis, clinical manifestations, diagnosis, and treatment of PBC are discussed elsewhere. (See "Clinical manifestations, diagnosis, and prognosis of primary biliary cholangitis (primary biliary cirrhosis)" and "Overview of the treatment of primary biliary cholangitis (primary biliary cirrhosis)" and "Pathogenesis of primary biliary cholangitis (primary biliary cirrhosis)" and "Trials of ursodeoxycholic acid for the treatment of primary biliary cholangitis (primary biliary cirrhosis)".)
OPTIMAL TIME FOR TRANSPLANTATION
An important issue is to determine the optimal time to perform a liver transplantation. Many groups have developed models that use clinical variables to estimate patient survival. Two types of models have been developed: one based upon initial data on entry into the study, and one that uses both initial and follow-up data. The Mayo model (table 1), which uses data from the initial evaluation, is most widely used, but because of individual patient variation, it does not replace the input of an experienced clinician [2,3]. The Mayo model can predict short- and long-term survival using current laboratory and clinical data. This tool can be used to anticipate liver failure, allowing the clinician to refer the patient for transplantation in a timely manner. Although these models are specific to survival in PBC, because the MELD score is used to prioritize patients for transplantation, it is often used to determine when to refer patients for transplantation (See "Model for End-stage Liver Disease (MELD)".)
In addition to considering the MELD score and Mayo model, we suggest that patients with PBC be referred for transplantation evaluation if one or more of the following is present:
●The plasma bilirubin concentration is greater than 5 mg/dL and is increasing
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- Personal communication from Marshall Kaplan, MD. He observed that five of six patients treated with ursodeoxycholic acid and colchicine developed normal serum transaminase and bilirubin levels and became asymptomatic.
- OPTIMAL TIME FOR TRANSPLANTATION
- Models based upon initial data
- Models based upon initial and follow-up data
- OUTCOME AFTER LIVER TRANSPLANTATION
- Effect of transplantation on symptoms
- Recurrence of PBC in the transplanted liver
- - Rate of recurrence
- - Clinical significance
- - Risk factors
- - Prevention
- - Diagnosis
- - Treatment
- INFORMATION FOR PATIENTS
- SUMMARY AND RECOMMENDATIONS