Primary biliary cholangitis (PBC), formerly
known as primary biliary cirrhosis, is
a chronic liver disease resulting from
progressive destruction of the bile ducts
in the liver – called the intrahepatic bile
ducts. Bile produced in your liver travels
via these ducts to your small intestine
where it aids in the digestion of fat and
fat-soluble vitamins (A, D, E and K). When
the ducts are destroyed, bile builds up
in the liver contributing to inflammation
and scarring (fibrosis). Eventually this
can lead to cirrhosis and its associated
complications, as scar tissue replaces
healthy liver tissue and liver function
becomes increasingly impaired.

What Causes PBC to Develop?
The exact cause of PBC is unknown. It is
not caused by alcohol or illegal-drug use.
It’s most likely an autoimmune disease
that occurs in genetically susceptible
individuals. The body’s immune system
mistakenly attacks and destroys its
own cells – in this case, the cells of the
intrahepatic bile ducts. Some people
diagnosed with PBC may also have one or
more other autoimmune diseases.
Who is at Risk for PBC?
• Women are nine times more likely than
men to develop PBC, meaning that
women make up about 90% of PBC cases.
• The disease most often develops
during middle age and is usually
diagnosed in people between the ages
of 35 to 60 years.
• There appears to be a genetic
component to developing PBC, as it’s
more common among siblings and in
families where one member has been
affected.
What are the Signs and
Symptoms of PBC?
PBC may progress slowly and many people
do not have symptoms, particularly in
the early stages of the disease. The most
common initial symptoms are fatigue
and itching of the skin (pruritus). Other
symptoms may include:
• Abdominal pain
• Darkening of the skin

• Small yellow or white bumps under the
skin (xanthomas) or around the eyes
(xanthelasmas)
Individuals may also complain of having
dry mouth and eyes, and bone, muscle and
joint pain.
As the disease progresses, symptoms of
cirrhosis can develop including:
• Yellowing of the skin (jaundice)
• Swelling of the legs and feet (edema)
• Enlarged abdomen from fluid
accumulation (ascites)
• Internal bleeding in the upper stomach
and esophagus from enlarged veins
(varices)
Thinning of the bones (osteoporosis)
leading to fractures is another
complication of PBC. While this is more
common in late stages of the disease, it can
occur earlier as well. In addition, people
with cirrhosis are at increased risk for liver
cancer (hepatocellular carcinoma).
How is PBC Diagnosed?
Because many people with PBC have no
symptoms, the disease is often discovered
incidentally due to abnormal results
on routine liver blood tests. Once PBC
is suspected, a blood test to check for
antimitochondrial antibody (AMA) is done.
This test is positive in nearly all people with
PBC. A liver biopsy, where a small sample of
liver tissue is removed with a small needle can
help confirm the diagnosis. Imaging studies
may be used to rule out other diseases, or
to further evaluate patients once they have
been diagnosed with PBC.
Primary Biliary Cholangitis 4
How is PBC Treated?
Treating the Disease
There is no cure for PBC, however, there
are medications that can help slow disease
progression and manage symptoms.
Ursodiol (brand names Actigall, URSO 250,
URSO Forte) is a naturally occurring bile acid
(ursodeoxycholic acid or UDCA) that helps
move bile out of the liver and into the small
intestine. If used early enough, Ursodiol can
improve liver function and may keep you
from needing, or delay the need for a liver
transplant. People with PBC must take this
medication every day for life.
UDCA is effective in more than 50 percent
of patients, but up to 40 percent of patients
do not achieve an adequate reduction in
alkaline phosphotase (ALP) or bilirubin
with UDCA, while 5-10 percent are unable
to tolerate UDCA.
In May 2016, obeticholic acid (brand name
Ocaliva) was approved for the treatment of
PBC in combination with UDCA in adults
with an inadequate response to UDCA, or as
a single therapy in adults unable to tolerate
UDCA. Obeticholic acid increases bile flow
from the liver and suppresses bile acid
production in the liver, thus reducing the
exposure of the liver to toxic levels of bile
acids. Side effects of obeticholic acid may
include increased itching and elevations in
blood lipids.
Other alternative therapies in patients
who are incomplete responders to UDCA
include fenofibrate. Medications to
5 Primary Biliary Cholangitis
suppress the immune system may also
be prescribed including prednisone or
azathioprine in PBC patients with the
“overlap syndrome” with autoimmune
hepatitis. Liver transplantation is
considered when medical treatment no
longer sufficiently controls the disease.
When a person has end-stage liver disease,
a liver transplant is necessary for survival.

Treating the Symptoms
• Intense itching is one of the most
common symptoms of PBC. Overthe-counter
antihistamines like
diphenhydramine (Benadryl) may
be helpful. Other agents such as
rifampicin, naltrexone, cholestyramine
and sertraline may be prescribed.
• Dry eyes can be relieved by using eye
drops (artificial tears).
• A dry mouth may be helped by sucking
on hard candy or chewing gum,
both of which increases saliva. There
are also saliva substitutes and some
medications that can be used.
Preventing Complications
• Blood tests to monitor for deficiencies in
fat-soluble vitamins are often done. As
PBC progresses, some people need to
replace the fat-soluble vitamins lost in
fatty stools, so you may be put on vitamin
A, D, E and K replacement therapy.
• Since people with PBC are at a higher risk
for osteoporosis, calcium and vitamin D
are usually prescribed. A baseline bone
density is currently recommended at the
time of initial diagnosis.
Primary Biliary Cholangitis 6
• As the ability of the liver to function
decreases over time, complications
associated with cirrhosis will need to
be addressed and treated. Screening
for varices and liver cancer is often
recommended.
What Lifestyle Changes are
Recommended for People
with PBC?
Maintaining a healthy lifestyle can help
people with PBC feel better, as well
as relieve or prevent some symptoms
associated with the disease. Upon
diagnosis, your doctor may suggest the
following:
• Start a reduced sodium diet if you have
fluid overload (edema or ascites)
• Reduce fat intake if you are overweight or
have fatty liver
• Drink plenty of water
• Avoid or lower intake of alcohol
• Avoid undue stress when possible
• Start exercising, particularly walking
• Stop smoking
• Maintain good skin care
• Get regular dental examinations
Keep in mind that PBC usually advances
slowly over a period of years. Many
people lead normal lives for years
without symptoms, depending on how
early the diagnosis is made. And while
there is no cure, people are having good
results slowing disease progression and
living longer without complications by
adhering to their medication regimen and
maintaining a healthy lifestyle.

http://www.liverfoundation.org/downloads/alf_download_1300.pdf