Primary Biliary Cirrhosis (PBC)
Primary Biliary Cirrhosis is an
autoimmune of the liver that slowly and progressively
destroys the small bile ducts which causes bile to
build up in the liver and bloodstream. Bile is the
fluid that your liver produces to help digest fats
and rid your body of other toxins. With PBC there
is no where for the bile to go so it backs up into
your Liver causing the damage and resulting in the
break-down of the entire process.
Initial symptoms of Primary Biliary Cirrhosis might
include fatigue, itchy skin, jaundice, fluid retention
in the abdomen and esophageal problems. PBC has is
also commonly seen with other autoimmune diseases
such as Rheumatoid Arthritis, CREST Syndrome, Hypothyroidism,
Raynaud's Phenomenon and Sjogren's Syndrome.
Diagnosing PBC can be difficult since it is a slow
and progressive disease, and many of the blood tests,
symptoms and imaging results mimic other conditions.
Symptoms:
Fatigue
Itchy skin
Jaundice
Yellow colored growth on the skin
Abdominal Pain
Enlarged Liver
Fatty Stools
Diagnosis:
Diagnosing PBC usually begins as an exclusion of
other conditions with similar symptoms and results,
such as hepatitis or primary sclerosing cholangitis.
Treatment for these other conditions may even be tried
as a way to rule out other conditions.
Imaging such as abdominal ultrasounds, CT Scans and
MRI's are often used, tissue biopsies may be taken
and blood tests may also show:
Positive Anti-Mitochondrial Antibody
Positive Anti-Nuclear Antibody (ANA)
Elevated Aspartate Transaminase
Elevated Aldolase
Elevated Alanine Transaminase
There are 4 stages to the progression of PBC (credited
to Wikipedia.org)
Stage 1 - Portal Stage: Normal
sized triads; portal inflammation, subtle bile duct
damage. Granulomas are often detected in this stage.
Stage 2 - Periportal Stage: Enlarged
triads; periportal fibrosis and/or inflammation. Typically
characterized by the finding of a proliferation of
small bile ducts.
Stage 3 - Septal Stage: Active
and/or passive fibrous septa.
Stage 4 - Biliary Cirrhosis: Nodules
present; garland
Treatment:
There is no cure for PBC, but treatment is available
that can slow the progression of the disease. The
most commonly used medication is Ursodeoxycholic acid
which can improve the removal of the bile from the
bloodstream. Cholestyramine (or colestipol) may also
be used to help relieve itchy skin.
Lifestyle adjustments may also be required such as
abstaining from alcohol as it can speed up the progression
of the disease. Multivitamins are also recommended.
Prognosis:
With effective treatment, progression can be slowed,
but never cured or stopped completely. As long as
patients take the treatments and lifestyle adjustments
recommended, most PBC sufferers can live near-normal
lives for quite some time. It is very important that
patients have their livers regularly monitored by
their physicians and watch for signs of worsening
symptoms.
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