Primary Biliary Cirrhosis (cont.)
In this Article
Symptomatic phase
This phase is defined by a positive AMA, persistently abnormal liver blood
tests, and the presence of symptoms of PBC. The duration of this phase among
patients is also quite variable, lasting from 3 to 11 years.
Advanced phase
In this phase, symptomatic patients develop the complications of cirrhosis
and progressive liver failure. The duration of this phase ranges from months to
2 years. These patients are at risk of dying unless they undergo successful
liver transplantation.
What is the role of mathematical models in predicting the outcome (prognosis) in PBC?
Investigators at the Mayo Clinic performed statistical analyses of many variables (different
types of data) among a large group of patients with PBC followed for
many years. They used the results to derive a mathematical equation to calculate
a so-called Mayo Risk Score (MRS). It turns out that the calculation is
based on the results of three of the patient's blood tests (total bilirubin,
albumin, and prothrombin time), the age of the patient, and the presence
of enough fluid retention to swell the legs (edema) or abdomen (ascites). The Mayo
Risk Score provides accurate information about the outcome (prognosis) of
individual patients over time. It has been validated and is currently used to
determine which patients with PBC need to be put on a liver transplant waiting
list.
Physicians can rather easily calculate a Mayo Risk Score for their patients
by going to the Internet site of the Mayo Clinic. There is no charge. The
results provide an estimated survival for the patient over the next several
years. Patients with an estimated life expectancy of 95% or less over one year
meet the minimal listing criteria set by the United Network of Organ Sharing (UNOS)
for liver transplant candidates.
What about pregnancy in PBC?
As discussed earlier, some women experience itching during their last
trimester of pregnancy when the hormone levels of estrogens are high. A minority
of these women may have a predisposition to develop PBC or may actually have
early PBC that has not yet been diagnosed.
In the medical literature, pregnancy in women with an established diagnosis
of PBC has not been reported frequently. While early reports suggested that the
outcome was suboptimal for both fetus and mother, later reports indicated that
women with PBC can deliver healthy babies. However, these women may develop
itching or jaundice during the last trimester. Otherwise, the clinical course of
PBC does not tend to worsen or improve during most pregnancies. Although some
babies may be born several weeks prematurely, only one miscarriage has been
reported. Furthermore, the risk of fetal abnormalities does not appear to
increase in the pregnancies of PBC women.
Since advanced cirrhosis interferes with the processing (metabolism) of sex
hormones, the likelihood of a woman with advanced liver disease becoming
pregnant is small. Nevertheless, it is important to know that PBC patients who
might become pregnant should not receive injections of vitamin A because it can
cause birth defects (See the section on treatment of fat malabsorption). The
chance that ursodeoxycholic acid therapy causes fetal harm is classified as
remote but possible since adequate studies have not been done in pregnant women.
The safety of ursodeoxycholic acid therapy taken by PBC mothers for their
breast-feeding infants is unknown and considered controversial.
Last Editorial Review: 9/17/2005
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