is an
Autoimmune Disease of the
Liver marked by the slow progressive destruction of the small bile ducts within the liver. When these ducts are damaged
Bile builds up in the liver (
Cholestasis ) and over time damages the tissue. This can lead to scarring,
Fibrosis ,
Cirrhosis , and ultimately
Liver Failure . It is a rare disease, about 200 out of a million; 10 to 1 women to men, although different references vary widely on these numbers.
The following signs may be present in PBC:
To diagnose PBC, distinctions should be established from other conditions with similar symptoms, such as
Autoimmune Hepatitis or
Primary Sclerosing Cholangitis (PSC).
Diagnostic
Blood Test s include:
Abdominal
Ultrasound or a
CT Scan is usually performed to rule out blockage to the bile ducts. Previously most suspected sufferers underwent a
Liver Biopsy , and - if uncertainty remained -
Endoscopic Retrograde Cholangiopancreatography (ERCP, an
Endoscopic investigation of the
Bile Duct ). Now most patients are diagnosed without invasive investigation since the combination of anti-mitochondrial antibodies (see below) and typical (cholestatic) liver function tests are considered diagnostic.
The cause of the disease is unknown at this time, but research indicates that there is an
Immunological basis for the
Disease , making it an
Autoimmune Disorder . Most of the patients (>90%) seem to have auto-mitochondrial antibodies (AMAs) against
Pyruvate Dehydrogenase complex (PDC-E2), an enzyme that is found in the
Mitochondria .
There is no known cure, but medication may slow the progression so that a normal lifespan and quality of life may be attainable for many patients. However, specific treatment for fatigue, which may be invalidating in some patients, is unavailable.
Ursodeoxycholic Acid (
Ursodiol ) is one, which helps reduce the cholestasis. To relieve itching caused by bile acids in circulation, which would normally be removed by the liver,
Cholestyramine (a
Bile Acid Sequestrant ) may be prescribed to absorb bile acids in the gut and be eliminated, rather than re-enter the blood stream. As in all liver diseases,
Alcoholic Beverage s are contraindicated. In advanced cases, a
Liver Transplant , if successful, results in a favourable prognosis.
- M. Eric Gershwin, John M. Vierling, Michael P. Manns, eds. ''Liver Immunology''. Philadelphia, Pa.: Hanley and Belfus, 2003. ISBN 1560534990. (State of the art; technical.)
- Sanjiv Chopra. ''The Liver Book: A Comprehensive Guide to Diagnosis, Treatment, and Recovery'', Atria, 2002, ISBN 0743405854
- Melissa Palmer. ''Dr. Melissa Palmer's Guide to Hepatitis and Liver Disease: What You Need to Know'', Avery Publishing Group; Revised edition May 24, 2004, ISBN 1583331883. her webpage .
- Howard J. Worman. ''The Liver Disorders Sourcebook'', McGraw-Hill, 1999, ISBN 0737300906.
