Primary Biliary Cirrhosis (cont.)
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:
- 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 |
- Absence of symptoms
- Normal liver tests
- AMA positive
|
Poorly defined, estimated as 2 to 10 years |
| Asymptomatic |
- Absence of symptoms
- Abnormal liver tests
- AMA positive
|
Indefinite in some patients, 2 to 20 years in
others |
| Symptomatic |
- Symptoms
- Abnormal liver tests
- AMA positive
|
3 to 11 years |
| Advanced |
- Symptoms
- Complications of cirrhosis and liver failure
- Abnormal liver tests
- AMA positive
|
0 to 2 years, without liver transplant |
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