Primary Biliary Cirrhosis (cont.)
In this Article
Preclinical phase
The first phase is characterized by the presence of AMA at a titer of greater
than or equal to 1:40 in an adult without any abnormality of liver blood tests
or any symptoms of liver disease. This phase is referred to as preclinical
because there is usually no reason for people in this phase of the disease to
see a physician or have testing. Furthermore, since screening tests for AMA are
not routinely performed, only small numbers of such people have been identified.
So, people with an AMA without symptoms or abnormal liver blood tests have been
identified only as the result of research studies of autoantibodies in
apparently healthy people.
However, even with only the isolated positive AMA, these people do appear to
have PBC. This conclusion is based on the presence of diagnostic or compatible
features on a liver biopsy and subsequent findings or clinical events during
long-term observation. Thus, more than 80% of these individuals with only a
positive AMA ultimately develop cholestatic liver blood tests followed by the
typical symptoms of PBC.
After discovery of an isolated positive AMA test, the time before development
of cholestatic liver tests ranged from 11 months to 19 years. The median time
(the time at which 50% of the people had developed cholestatic liver tests) was
5.6 years. During 11 to 24 years of observation starting in the preclinical
phase of 29 patients, 5 died. However, none of the five died as a result of
liver disease and the median age at death was 78.
Asymptomatic phase
This phase is characterized by a positive AMA and cholestatic liver blood
tests in a person without symptoms of liver disease. The incidental discovery of
an elevated alkaline phosphatase is what most commonly leads to the diagnosis of
PBC in this phase. The elevated alkaline phosphatase is usually discovered after
testing the blood routinely or for another clinical reason.
The results of three large studies indicate that 40% of these asymptomatic
patients will develop symptoms of liver disease within the next 6 years. Over
and above that, another 33% of patients will likely develop symptoms between 6
and 12 years. Longer follow up is not available, but this asymptomatic phase may
persist indefinitely in a minority of patients with PBC.
Next: Symptomatic »
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