Primary Biliary Cirrhosis (cont.)
In this Article
What manifestations are specifically due to PBC itself?
The following manifestations (symptoms and findings) due to PBC will be discussed:
Fatigue
The most common symptom of PBC is fatigue, which occurs in up to 70% of
patients. The presence and severity of fatigue, however, does not correspond
(correlate) with the severity of the liver disease. It should be noted that
significant fatigue can be either the cause or the result of difficulty sleeping
or depression.
Fatigue associated with inflammation of the liver is often characterized by
normal energy during the initial half to two thirds of the day followed by a
profound loss of energy that requires rest or a substantial reduction in
activity. Thus, when patients report being exhausted in the morning, it is
likely that sleep deprivation and depression are the cause of the exhaustion
rather than PBC. Most people with PBC report that a nap does not rejuvenate
them. Conversely, many PBC patients inexplicably experience occasional days
without a loss of energy.
In summary, the main characteristics of fatigue due to liver inflammation in
PBC are:
- Fatigue is often absent in the morning
- Rapid decrease in energy later in the day
- Failure to rejuvenate with a rest period
- Occasional days without fatigue
Itching
Just about as common as fatigue in PBC, itching (pruritus) of the skin
affects 66% of patients at some time during the disease. The itching tends to
occur early in the course of the disease, when patients still have good liver
function. As a matter of fact, itching can even be the initial symptom of PBC.
It is interesting to note that some women with PBC experienced itching during
the last trimester (three months) of a prior pregnancy, before they knew about
their PBC. In a condition called cholestasis of pregnancy, some otherwise normal
women during the last trimester develop cholestasis and itching that resolve
following delivery. (Remember that cholestasis means decreased bile flow.) Of
course, most women with cholestasis of pregnancy do not go on to develop PBC.
Yet, it turns out that about 5% of women diagnosed with PBC give a history of
having had such itching during a prior pregnancy.
Characteristically, the itching in PBC begins in the palms of the hands and
soles of the feet. Later, it may affect the entire body. The intensity
fluctuates in a circadian rhythm, meaning that the itching can worsen at night
and improve during the day. Nocturnal itching can disrupt sleep and lead to
sleep deprivation, fatigue, and depression. Rarely, the itching is so severe and
unresponsive to therapy that the person may become suicidal. Prolonged itching
and scratching causes scratch marks (excoriations), thickening, and darkening of
the skin.
The cause (etiology and pathogenesis) of itching remains unclear. The bile
acids, as previously mentioned, normally are transported in bile from the liver,
through the bile ducts, to the intestine. Most of the bile acids are then
reabsorbed in the intestine and go back to the liver for reprocessing and
recycling. In cholestasis, therefore, the bile acids back up from the liver,
accumulate in the blood, and, for some years, were presumed to be the cause of
the itching. Modern studies, however, have just about refuted the notion that
the itching in PBC and other cholestatic liver diseases is caused by bile acids.
Recently, the itching was considered (postulated) to be due to accumulation
of an endorphin, a natural substance that attaches (binds) to the natural
receptors (acceptors) for morphine in nerves. You see, nerves in the skin carry
the sensation of itching. Indeed, the finding that itching improved in some
people treated with drugs that block the binding of morphine or endorphins to
nerves supported this consideration. Yet, many patients do not respond to these
blocking drugs, suggesting that other causes or mechanisms are involved in
producing itching.
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