Primary Biliary Cirrhosis (cont.)Medical Author:
John M. Vierling, MD, FACP
John M. Vierling, MD, FACPJohn 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. Medical Editor:
Leslie J. Schoenfield, MD, PhD
Leslie J. Schoenfield, MD, PhDDr. 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. In this Article
 What are the criteria for a definitive diagnosis of PBC?The criteria for a definitive diagnosis of PBC were established for the purpose of conducting clinical research, including therapeutic trials, on the disease. The criteria were designed to identify all patients with classic PBC and exclude any patient with a questionable diagnosis. A definitive diagnosis of PBC is established in a patient who has all three of the following:
What is the course of natural progression in PBC?The course of natural progression (the natural history) in PBC can be divided into four clinical phases (preclinical, asymptomatic, symptomatic, and advanced). What's more, based on our knowledge of the clinical findings in patients with PBC, mathematical models have been developed that can predict the outcome (prognosis) for individual patients. What are the sequential clinical phases of PBC?The four sequential clinical (symptoms and tests) phases of PBC are: It is important to realize that the time required to evolve from one clinical phase to another varies substantially among individuals. Also, be aware that these clinical phases are different from the pathological stages determined by the liver biopsy. Most importantly, since the diagnosis is often first made between the ages of 30 and 60 years and progression of the disease is usually so slow, PBC does not result in a reduced life expectancy in all patients. Table 3 shows the sequential phases in the natural progression of PBC without therapy.
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Primary Biliary Cirrhosis - Symptoms
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