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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

Rheumatoid arthritis

An abnormal type of antibody, called rheumatoid factor, is found in the blood of most patients with rheumatoid arthritis. This antibody also is found, however, in approximately 25% of patients with PBC. Although some PBC patients with the rheumatoid factor also have symptoms of joint pain and stiffness, most do not.

Celiac sprue

This autoimmune disease of the gut occurs in about 6% of patients with PBC. The disease impairs intestinal absorption of dietary fat and other nutrients, resulting in diarrhea and nutritional and vitamin deficiencies. Celiac sprue is caused by intolerance to gluten, a component of wheat, barley, and rye in the diet. As already mentioned, similar symptoms can occur in PBC itself as a result of fat malabsorption due to decreased bile flow into the gut. In any case, PBC patients with the signs or symptoms of fat malabsorption should be tested for celiac sprue. The diagnosis of celiac sprue is made by finding certain serum antibodies (for example, those called antigliadin or antiendomysial antibodies), characteristic intestinal biopsy features, and a usually dramatic response to dietary restriction of gluten.

Urinary tract infections

Recurrent bacterial infections of the urine occur in 19% of women with PBC. These infections may be without symptoms or cause a sense of a frequent, urgent need to urinate with a burning feeling while passing urine.

Gallstones

Patients with PBC can develop two types of gallstones in the gallbladder. One type (called cholesterol gallstones) contains mostly cholesterol, and is by far the most common type of gallstone found in the general population. The other type (called pigment gallstones) contains mostly bile pigments (including bilirubin) and calcium. This type of gallstone occurs with increased frequency in all types of cirrhosis, including PBC.

Gallstones occur in about 30% of adults in the general population and are at least twice as common in women as in men. It is not surprising, therefore, that gallstones are especially frequent in individuals having other conditions that tend to afflict women more than men, such as PBC. The most common symptom of gallstones is abdominal pain. Sometimes, they can cause nausea, fever, and/or jaundice. But the majority of gallstones do not cause any symptoms. The diagnosis of gallstones is usually made by ultrasound imaging of the gallbladder.