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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

PBC is a disease that disproportionately affects women, with 10 women for every man having the disease. It is also a disease of adulthood that, rather curiously, has never been diagnosed in childhood. As a matter of fact, the diagnosis is made most frequently in middle-aged people, between the ages of about 30 and 60 years. PBC is considered to be an uncommon disease, but not rare. Studies indicate that the number of people with PBC at a given time (referred to as the prevalence of disease) ranges from 19 to 251 per million population in various countries. If these figures are adjusted to compensate for the fact that PBC is found only in adults and that 90% of the patients are women, then the calculated prevalence is approximately 25 to 335 per million women and 2.8 to 37 per million men.

The largest and best long-term studies of PBC have been conducted in northern England. Their findings indicate that the number of new cases of PBC over time (referred to as the incidence of disease) has increased steadily from 16 per million population in 1976 to 251 per million in 1994. Unfortunately, no similar studies have been conducted elsewhere to validate or refute the belief that the incidence and prevalence of PBC is rising worldwide.

One comprehensive study conducted in the north of England from 1987 to 1994 was designed specifically to find people with PBC. Using strict criteria for the diagnosis of PBC, they identified a total of 770 patients. Of these, the number of newly diagnosed people with PBC during just these 7 years was 468. Thus, clinical investigators interested in PBC had conducted extensive epidemiological (cause and distribution) studies of PBC over almost 20 years in this same geographic area. Such a concentrated focus of effort strongly supports the view that the apparent increase in the number of people with PBC is indeed a true increase.