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PRIMARY BILIARY CIRRHOSIS
Primary biliary cirrhosis
(PBC) is a chronic liver disease that causes slow, progressive destruction
of bile ducts in the liver. This destruction interferes with the excretion
of bile. Continued liver inflammation causes scarring and eventually leads
to cirrhosis.
Cirrhosis is present only in the
later stage of the disease. In the early stages of the illness, the main
problem is the build up of substances (like acids, cholesterol) in the
blood, which are normally excreted into the bile.
PRESENTATION
Women are affected
10 times more frequently than men. The disease usually is first diagnosed
in people 30 to 60 years old. Many patients have no symptoms of disease
and are diagnosed by finding an abnormality on routine
liver blood tests. Itching and fatigue are common symptoms. Other
signs include jaundice, cholesterol deposits in the skin, fluid accumulation
and darkening of the skin. Several other disorders are often associated
with PBC. The most common are impaired functioning of the tear and salivary
glands, causing dry eyes or mouth. Arthritis and thyroid problems may also
be present. Bone softening and fragility leading to fractures can occur
in late stages of the disease.
DIAGNOSIS
PBC diagnosis is based
on several pieces of information. The patient may have symptoms (itching)
suggesting bile duct damage. Laboratory tests, such as the alkaline phosphatase
activity test, may confirm this. The test for mitochondrial antibodies
is particularly useful as it is positive in nearly all patients. Often,
the bile ducts are X-rayed to rule out possibilities of other causes
of biliary tract disease, such as obstruction. A liver biopsy is useful
in confirming the diagnosis and in giving information on the severity and
extent of liver damage.
CAUSE
Although the cause
of the initial bile duct damage in PBC is unknown, there are certain clues
that may be important. Strictly speaking, the disease is not inherited,
but it is more common among siblings and in families where one member
has previously been affected. Multiple disturbances of the immune system
have been found in persons with PBC and may be an important factor. Hormones
may also play a role given that this illness is so much more common in
women.
PROGNOSIS
PBC advances slowly.
Many patients lead active and productive lives for ten to fifteen years
after diagnosis. Patients who show no symptoms at the time of diagnosis
often remain symptom free after ten years. Jaundice appears to be a sign
of diminishing liver reserve and may be an important indication of the
progress of the disease. The illness is chronic and may lead to life threatening
complications, especially after cirrhosis develops.
TREATMENT
Treatment may be useful
in several ways. Proper advice will ensure the elimination of potentially
harmful drugs, foods or toxins. If the patient is deficient in vitamin
D, then this should be corrected. The thyroid function should be tested
and if low, treated with a thyroid hormone. Symptoms may be successfully
relieved. Itching is often reduced by using cholestryramine and rifampin.
Salt restriction may be effective in reducing fluid accumulation. The diet
should be well balanced. Corticosteroids have been found ineffective in
most patients. Few drugs such as Actigall and methotrexate have shown
some promise in improving liver tests, but their impact on the survival
rate remains unclear. Colchicine improves liver tests but does not slow
the progression of the disease.
LIVER TRANSPLANTATION
When medical treatment
no longer controls the disease and the patient has severe liver failure,
liver transplantation is indicated. Signs of
liver failure include accumulation of fluid in the abdomen, malnutrition,
gastrointestinal bleeding, intractable itching, jaundice, and bone fractures.
Transplantation may be recommended before all these events occur. The outcome
for patients with PBC who have undergone transplantation is excellent.
The survival rate for two or more years is about 60-80 percent. The use
of new drugs to suppress rejection has made transplantation even more successful. The disease's slow progress makes it possible to plan elective transplant
surgery.
THE FUTURE
PBC has been known
for more than 100 years. This knowledge has led doctors to make earlier
diagnoses. Many clues to the cause have been supplied by careful observation
of patients over the last 25 years, but the basic cause is unknown. Research
is following two paths:
-
Basic investigation of the causes and development
of the disease.
-
Drug therapy trials, involving a large number of
patients around the world, are exploring the potential use of several medications
to lessen the symptoms and control liver damage.
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