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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 - Symptoms
• Patient Comments: Primary Biliary Cirrhosis - Diagnosis
• Find a local Gastroenterologist in your town
• Primary Biliary Cirrhosis Index

Other associated diseases

Rarely, an inflammatory bowel disease (ulcerative colitis or Crohn's disease), a kidney problem (renal tubular acidosis), poor pancreatic function (pancreatic insufficiency), as mentioned earlier, or a lung condition (pulmonary interstitial fibrosis) can be associated with PBC.

Identification of risk factors for developing PBC should be an important priority, but astonishingly little research has been done in this area. A recent survey used questions from the National Health and Nutrition Examination Survey of the U.S. government. This survey compared the answers of 199 patients with PBC with those of 171 siblings and 141 friends of the patients. As anticipated, the patients with PBC were predominantly women (10 to 1 women to men), and the average age was 53 years.

The patients reported having had a high frequency of other autoimmune diseases, including sicca syndrome in 17.4% and Raynaud's phenomenon in 12.5%. Interestingly, 6% reported that at least one other family member had PBC. Statistical analysis showed that the risks of developing PBC for patients compared to friends as controls were:

• 492% greater for having had other autoimmune diseases
• 204% greater for having smoked cigarettes
• 186% greater for having had tonsillectomy
• 212% greater among women for having had urinary tract infections or vaginal infections.

Similar increased risks were found for the PBC patients when they were compared to siblings without PBC.

The diagnosis of PBC is established by the results of several types of tests. These include blood tests, serum autoantibody testing, ultrasound imaging of the liver, and liver biopsy.