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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

• Thyroid dysfunction
• Sicca syndrome
• Raynaud's phenomenon
• Scleroderma
• Rheumatoid arthritis
• Celiac sprue
• Urinary tract infections
• Gallstones
• Other associated diseases

Thyroid dysfunction

Up to 25% of patients with PBC develop an autoimmune reaction against the thyroid gland. This reaction results in an inflammation of the gland, called thyroiditis. When the thyroid gland is first inflamed, only a minority of these individuals experience thyroid tenderness or pain. This pain is usually mild and located over the gland in the front of the lower neck. In fact, most people do not experience symptoms from the thyroiditis until some months or years after the autoimmune reaction began. By then, the slow and gradual decrease in thyroid function resulting from the inflammation can cause an underproduction of thyroid hormone, called hypothyroidism.

It should be noted that the symptoms and signs of hypothyroidism, which include fatigue, weight gain, and elevated cholesterol, develop gradually and can be quite subtle. Further, they can easily be confused with those of PBC itself. Thus, physicians should periodically test thyroid function in all patients with PBC to detect hypothyroidism and to initiate treatment by replacement of thyroid hormone. Often, however, the thyroiditis occurs and the indications of hypothyroidism are found well before the diagnosis of PBC is made.