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Medical Author:

John M. Vierling, MD, FACP

John M. Vierling, MD, FACP

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.

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Medical Editor:

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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In this Article

• What is PBC?
• What is the scope of the problem?
• What is the cause of PBC?
• What are the symptoms and physical findings in PBC?
• What manifestations are specifically due to PBC itself?
• What are the manifestations of the complications of cirrhosis in PBC?
• What are the manifestations of diseases associated with PBC?
• What are risk factors for PBC?
• How is PBC diagnosed?
• What is the role of blood tests?
• What is the role of testing for antimitochondrial antibodies?
• What is the role of imaging tests?
• What is the role of liver biopsy?
• What are the criteria for a definitive diagnosis of PBC
• What is the course of natural progression in PBC?
• What are the sequential clinical phases of PBC?
• What is the role of mathematical models in predicting the outcome (prognosis) in PBC?
• What about pregnancy in PBC?
• Patient Comments: Primary Biliary Cirrhosis - Diagnosis
• Patient Comments: Primary Biliary Cirrhosis - Symptoms
• Find a local Gastroenterologist in your town
• Primary Biliary Cirrhosis Index

Symptomatic phase

This phase is defined by a positive AMA, persistently abnormal liver blood tests, and the presence of symptoms of PBC. The duration of this phase among patients is also quite variable, lasting from 3 to 11 years.

Advanced phase

In this phase, symptomatic patients develop the complications of cirrhosis and progressive liver failure. The duration of this phase ranges from months to 2 years. These patients are at risk of dying unless they undergo successful liver transplantation.

Investigators at the Mayo Clinic performed statistical analyses of many variables (different types of data) among a large group of patients with PBC followed for many years. They used the results to derive a mathematical equation to calculate a so-called Mayo Risk Score (MRS). It turns out that the calculation is based on the results of three of the patient's blood tests (total bilirubin, albumin, and prothrombin time), the age of the patient, and the presence of enough fluid retention to swell the legs (edema) or abdomen (ascites). The Mayo Risk Score provides accurate information about the outcome (prognosis) of individual patients over time. It has been validated and is currently used to determine which patients with PBC need to be put on a liver transplant waiting list.

Physicians can rather easily calculate a Mayo Risk Score for their patients by going to the Internet site of the Mayo Clinic. There is no charge. The results provide an estimated survival for the patient over the next several years. Patients with an estimated life expectancy of 95% or less over one year meet the minimal listing criteria set by the United Network of Organ Sharing (UNOS) for liver transplant candidates.