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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

Sicca syndrome

Up to 70% of patients with PBC experience a sensation of dry eyes or dry mouth referred to as sicca syndrome or alternatively, as Sjogren's syndrome. This syndrome is caused by an autoimmune inflammation of the lining cells of the ducts that carry tears or saliva. Rarely, patients experience the consequences of dryness in other areas of the body including the windpipe or larynx (causing hoarseness) and the vagina. This autoimmune inflammation and drying of secretions can also occur, although even more rarely, in the ducts of the pancreas. The resulting poor pancreatic function (pancreatic insufficiency) can cause impaired absorption of fat and the fat-soluble vitamins.

Raynaud's phenomenon

Raynaud's phenomenon starts with an intense blanching (paling) of the skin of the fingers or toes when they are exposed to the cold. When the hands or feet are re-warmed, the blanching characteristically changes to a purplish discoloration and then to a bright red, often associated with throbbing pain. This phenomenon is due to the cold causing a constriction (narrowing) of the arteries that supply blood to the fingers or toes. Then, with re-warming of the hands or feet, the blood flow is restored and causes the redness and pain. Raynaud's phenomenon is often associated with scleroderma. For more information about this phenomenon, please read the Raynaud's phenomenon. article

Scleroderma

Approximately 17% of patients with PBC develop mild scleroderma, a condition in which the skin around the fingers, toes, and mouth becomes tight. In addition, scleroderma involves the muscles of the esophagus and small intestine. The esophagus connects the mouth to the stomach, and its muscles help to propel swallowed food into the stomach. In addition, a band of muscle (the lower esophageal sphincter), which is located at the junction of the esophagus and stomach, has two other functions. One is to open to let food pass into the stomach. The other is to close to prevent the stomach juices that contain acid from flowing back into the esophagus.

Scleroderma, therefore, can also cause esophageal and intestinal symptoms. Thus, involvement of the esophageal muscles that propel food through the esophagus results in difficulty swallowing. Most often, patients experience this difficulty as a sensation of solid food sticking in the chest after swallowing. Involvement of the lower esophageal sphincter muscle prevents the closure of the lower end of the esophagus and thereby, allows reflux of stomach acid, causing the symptom of heartburn. The heartburn, which is not caused by a heart problem, is usually experienced as a sensation of burning in the center of the chest. Involvement of the muscles of the small intestine in scleroderma can cause a condition called bacterial overgrowth, which can lead to malabsorption of fat and diarrhea. For more about this condition, please read the Scleroderma article.

Finally, a minority of PBC patients has a variant of scleroderma referred to as CREST syndrome. The term CREST refers to Calcium deposits in the skin, Raynaud's phenomenon, muscle dysfunction of the Esophagus, tightening of the skin of the fingers called Sclerodactyly, and dilated small blood vessels beneath the skin called Telangiectasias.