Primary Biliary Cirrhosis Treatment (PBC)

  • Medical Author:

    John M. Vierling M.D. is Professor of Medicine and Surgery at the Baylor College of Medicine in Houston, Texas, where he also serves as Director of Baylor Liver Health and Chief of Hepatology. In addition, he is the Director of Advanced Liver Therapies, a center devoted to clinical research in hepatobiliary diseases at St. Luke's Episcopal Hospital. Dr. Vierling is board certified in internal medicine and gastroenterology and a Fellow of the American College of Physicians.

  • Medical Editor: Leslie J. Schoenfield, MD, PhD
    Leslie J. Schoenfield, MD, PhD

    Leslie J. Schoenfield, MD, PhD

    Dr. Schoenfield served as associate professor of medicine and consultant in gastroenterology on the faculty of the Mayo Clinic for seven years. He became a professor of medicine in residence at UCLA from 1972 to 1999 (now emeritus). He was the director of gastroenterology at Cedars-Sinai Medical Center in Los Angeles for 25 years, where he received the chief resident's teaching award, the president's award, and the pioneer of medicine award.

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What is the role of liver transplantation in PBC?

Liver transplantation is a life saving operation for those patients with PBC who are at risk of premature death due to liver failure or the complications of cirrhosis and portal hypertension. The Mayo Risk Score provides an accurate estimate of the future outcome (prognosis) for patients, regardless of whether they are being treated with ursodeoxycholic acid. The United Network for Organ Sharing (UNOS) permits patients with PBC to be listed for a liver transplant once their estimated survival with PBC for an additional year is 95% or less. It is recommended that physicians calculate the Mayo Risk Score at least yearly in all patients with PBC who have cirrhosis.

Most PBC candidates for transplantation have advanced cirrhosis with decompensated liver disease, which also is referred to as liver failure. Decompensated liver disease means that the patients have low levels of serum albumin and blood clotting factors made in the liver and complications of portal venous hypertension such as ascites, variceal bleeding, encephalopathy, or hypersplenism. Patients are classified as having decompensated cirrhosis whether or not the complications respond to medical therapy.

Rarely, before advanced cirrhosis develops, transplantation of the liver is warranted in a few clinically disastrous situations in PBC. Examples of such situations include recurrent fractures due to advanced osteoporosis or severe, debilitating itching unrelieved by any medical therapy, and even more rarely the hepatopulmonary syndrome (breathing difficulty in advanced cirrhosis). The regional review committees of UNOS accept applications for transplantation in these special circumstances and decide on a patient-by-patient basis if liver transplantation is indicated.

The outcome of liver transplantation in PBC is excellent. The survival of patients three to five years after transplantation is generally 80 to 90%. This impressive survival rate is greater than the survival of patients transplanted for most other types of liver diseases. The titers of AMA fall following successful liver transplantation, but they do not usually disappear. A minority of patients, however, develops recurrent PBC in the transplanted liver. Studies are being performed to see if reducing the maintenance doses of immunosuppressive drugs more slowly after transplantation can prevent recurrence of PBC. Once recurrent PBC is diagnosed, ursodeoxycholic acid therapy is started to retard progression. Patients with recurrent PBC rarely will need a second liver transplant.

Medically Reviewed by a Doctor on 9/25/2015

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