Primary Biliary Cirrhosis (cont.)
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.
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.
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?
- Find a local Gastroenterologist in your town
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:
- Cholestatic liver tests (alkaline phosphatase and ggt elevated more than ALT and AST)
- AMA positive at a titer of greater than or equal to 1:40
- Diagnostic or compatible liver biopsy
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.
| Phase | Characteristics | Duration |
| Preclinical |
|
Poorly defined, estimated as 2 to 10 years |
| Asymptomatic |
|
Indefinite in some patients, 2 to 20 years in others |
| Symptomatic |
|
3 to 11 years |
| Advanced |
|
0 to 2 years, without liver transplant |
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