Primary Biliary Cirrhosis
Primary biliary cirrhosis is a disease in which the bile ducts
in your liver are slowly destroyed. Your body has an intricate
system of ducts designed specifically to transport bile, a fluid
produced in your liver. Bile is essential for the proper
digestion of fats and helps rid your body of worn-out red blood
cells, cholesterol and potentially toxic metals. In primary
biliary cirrhosis, the destruction of your bile ducts can cause
harmful substances to build up in your liver and sometimes lead
to irreversible scarring of liver tissue (cirrhosis).
The cause of primary biliary cirrhosis remains unclear. Many
experts consider primary biliary cirrhosis an autoimmune disease
in which the body turns against its own cells, although it's
likely that genetic and environmental factors also play a part.
Primary biliary cirrhosis develops slowly. Medication can slow
the progression of the disease, especially if treatment begins
Signs and Symptoms
Although some people with primary biliary
cirrhosis remain symptom-free for years after they're diagnosed,
others experience fatigue, itching, dry eyes and dry mouth early
in the disease:
This is the most common symptom of primary biliary
cirrhosis. In general, energy levels are normal in the
morning, but fall later in the day and don't improve with
rest. Doctors haven't found any correlation between the
degree of exhaustion and the severity of the illness. This
means that people with mild primary biliary cirrhosis and
those with more serious disease may be equally fatigued.
Another common symptom, itching (pruritis), is often most
bothersome over your legs, arms and back. The severity of
itching may change, often becoming worse at night and
improving during the day. Nighttime itching can disturb
sleep, making fatigue worse and sometimes leading to
depression. The cause of this severe itching isn't clear.
Dry eyes and mouth (sicca
Sicca syndrome often occurs in people with other autoimmune
disorders. It causes inflammation in the moisture-secreting
glands of the eyes and mouth, resulting in the decreased
production of tears and saliva. This can lead to difficulty
swallowing, light sensitivity and corneal ulcers.
As the destruction of bile duct and liver cells
progresses, other signs and symptoms may develop, such as:
A common sign of advanced liver disease, jaundice turns your
skin and the whites of your eyes yellow. The discoloration
is due to high blood levels of bilirubin, a byproduct of the
breakdown of the hemoglobin from old or damaged red blood
cells. Normally, bile carries bilirubin out of the liver so
that it can be excreted from your body. But as more bile
ducts are destroyed and the flow of bile slows, bilirubin
begins to build up in the blood and eventually becomes
visible in your skin and eyes.
Inadequate bile flow increases the production of the skin
pigment melanin. This causes your skin to become darker,
even in areas that aren't exposed to the sun. Sometimes the
deeper color isn't uniform, and your skin appears blotchy.
Swollen feet (edema)
and abdomen (ascites).
As liver damage progresses, your body begins to retain salt
and fluids. At first, the excess water accumulates mainly in
your feet and ankles, which tend to become more swollen late
in the day. In time, fluid can also collect in your abdomen.
Cholesterol deposits (xanthomas).
Your body uses bile as the main way of eliminating excess
cholesterol. When disease interferes with this process, the
amount of cholesterol in the blood increases. This can lead
to the formation of fatty deposits in the skin around the
eyes, the eyelids, or in the creases in the palms, soles,
elbows, knees or buttocks. These raised, waxy growths
usually don't appear until blood cholesterol reaches very
high levels. Even then, not everyone with primary biliary
cirrhosis develops them.
Because bile is essential for the digestion and absorption
of fats, primary biliary cirrhosis can cause intestinal
problems. These include diarrhea and steatorrhea — greasy,
bad-smelling stools that result from poor fat digestion.
About one in five women with primary biliary cirrhosis
experiences recurring urinary tract infections. The
relationship between these infections and bile duct
destruction isn't well understood. A few studies suggest
that the same bacteria responsible for urinary tract
infections may play a role in some cases of primary biliary
The exact cause of primary biliary cirrhosis
isn't known, but it appears to be an immune system disorder that
slowly destroys the bile ducts in your liver. Genetics and the
environment also may play a role in this disease.
The Importance of Bile
Bile is a greenish-yellow fluid produced in the
cells of the liver. It's essential for digesting and absorbing
fats and is one of the main avenues for eliminating drugs,
cholesterol and metabolic byproducts from the body. Within the
liver, bile is excreted into canal-like spaces between the liver
cells, which drain into an interconnected series of thin tubes
(ducts). The initial ducts are quite small, but become
progressively larger as they spread through the liver, much like
the branches of a tree.
Bile leaves the liver through the common hepatic
duct — the "trunk" of the biliary tree — and enters the
gallbladder, a small, pear-shaped organ just below the liver,
through the cystic duct. The gallbladder stores bile in a highly
concentrated form until you eat, when it's released into the
common bile duct. From there, bile flows to the upper part of
the small intestine (duodenum), where it begins to break down
the fat in food.
A slow attack on your bile ducts
Most of the bile that enters the small intestine
is returned to the liver for reuse. Ordinarily, nearly a quart
of bile passes from the liver to the intestine every day.
Primary biliary cirrhosis destroys the small ducts within the
liver, slowing and sometimes blocking the normal flow of bile.
The problem begins with inflammation in the
smallest ducts in the liver. In time, the inflammation spreads
to and destroys nearby liver cells. As these cells are
destroyed, they're replaced by scar tissue (fibrosis). Over a
period of years, the combination of ongoing inflammation,
scarring and toxicity from trapped bile can lead to cirrhosis.
Cirrhosis involves irreversible scarring of liver tissue that
makes it impossible for the liver to carry out essential
immune system disorder?
Most evidence suggests that primary biliary
cirrhosis is an immune system disorder. The initial inflammation
begins with T lymphocytes (T cells) — white blood cells that
normally recognize and help defend against bacteria and fungi. T
cells invade and destroy the epithelial cells lining the small
bile ducts. The T cells also produce chemicals that stimulate
the epithelial cells to secrete proteins that attract more T
cells, thereby creating an ongoing cycle of damage.
This is a classic autoimmune scenario — a case in
which the body's immune system turns against its own cells. But
not everything about primary biliary cirrhosis is classic. For
one thing, it rarely occurs in children, even though autoimmune
disorders often begin in childhood. For another, unlike most
autoimmune diseases, primary biliary cirrhosis doesn't usually
respond to drugs that suppress the immune system. For these and
other reasons, researchers suspect that other factors play a
part in the disease, such as:
Primary biliary cirrhosis isn't transmitted from parent to
child and so isn't considered a hereditary disease. Yet
because it seems to run in families, researchers suspect
that some people may inherit certain immune system defects
that make them more susceptible to the disorder. Other
immune system genes may play a role in disease progression.
For decades, researchers have suspected that primary biliary
cirrhosis might result from a bacterial, fungal or parasitic
infection. That would explain the massing of T cells in the
small bile ducts and why the disease has some anomalies that
distinguish it from other autoimmune disorders. But no
commonplace infections have been linked to primary biliary
cirrhosis. Scientists are now focusing on retroviruses, the
same type of virus that causes HIV/AIDS.
Screening and Diagnosis
Many people with primary biliary cirrhosis have
no symptoms of the disease when they're diagnosed. Instead,
doctors often become aware of a problem during routine blood
tests or an evaluation for another condition.
Several tests can help diagnose primary biliary
Liver function tests.
These blood tests check the levels of enzymes that may
indicate liver disease in general and bile duct injury in
particular. Certain liver enzymes are elevated in most
people with primary biliary cirrhosis, especially alkaline
phosphatase, which is produced in the bile ducts.
This noninvasive test uses high-frequency sound waves to
create precise images of structures within the body,
including the bile ducts. It's sometimes used to rule out
other causes of bile flow blockage, such as gallstones or
Found in every cell, mitochondria are the prime energy
producers of the body. Antibodies are proteins in the blood
that help destroy bacteria and other harmful pathogens. Most
people with primary biliary cirrhosis have
anti-mitochondrial antibodies — antibodies that target
enzymes in the mitochondria. These antibodies almost never
occur in people who don't have primary biliary cirrhosis,
even if they have other liver disorders. For that reason, a
positive AMA test is considered a very reliable indicator of
the disease. At the same time, a small percentage of people
with primary biliary cirrhosis don't have AMAs.
False-positive tests, which indicate a problem where none
exists, also can occur. Because an AMA test isn't entirely
foolproof, doctors usually perform a liver biopsy, which can
definitively confirm the presence or absence of the disease.
In this test, a small sample of liver tissue (biopsy) is
removed and examined in a laboratory, either to confirm the
diagnosis or to determine the extent (stage) of the disease.
Doctors withdraw the tissue through a small incision using a
thin needle. Doctors may take more liver biopsies as time
goes on to check the progression of the disease.
resonance elastography (MRE).
This relatively new test can help your doctor diagnose
primary biliary cirrhosis and may help avoid the need for
liver biopsy, which is more invasive. MRE technology works
by combining traditional magnetic resonance imaging (MRI)
with low-frequency sounds waves. The MRI component uses a
magnetic field and radio waves to create clear and detailed
cross-sectional images of your body, which show size and
structure of your tissues and organs. The low-frequency
sound waves then help reveal physical properties of those
tissues and organs such as tissue stiffness. Stiffness of
your liver may indicate cirrhosis.
In some cases, doctors may be able to discover if a person is at
risk of primary biliary cirrhosis early. A 2007 study showed
that first-degree relatives of those with primary biliary
cirrhosis are more likely to have antibodies in their blood that
increase their risk of the disease. If you have a first-degree
relative who has primary biliary cirrhosis, your doctor can
screen your blood for these antibodies. By knowing whether you
have them, your doctor can more closely monitor you and may be
able to provide treatment earlier, should you develop the
disease. Early treatment is important and can help slow the
progression of the disease.
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