is an
Autoimmune Disease of the
Liver marked by the slow progressive destruction of the small bile ducts (
Bile Canaliculi ) 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 was previously thought to be a rare disease, but more recent studies have shown that it may affect up to 1 in 3-4,000 people; the sex ratio is 10 to 1, women to men.
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. However, a liver biopsy is necessary to determine the stage of disease.
appear to be prognostic agents in PBC.
Anti-glycoprotein-210 Antibodies , and to a lessor degree
Anti-p62 Antibodies correlate with progression toward end stage liver failure.
Anti-centromere Antibodies correlate with developing portal hypertension.
1. Anti-np62
2 and anti-sp100 are also found in association with PBC.
- ''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 Septae .
- ''Stage 4 - Biliary Cirrhosis'': Nodules present; garland or jigsaw pattern.
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 complex that is found in the
Mitochondria . In addition, a more specific test to confirm this disease from a bone disorder such as Paget's (disease of bone) which also has increases in Alkaline phosphatase is the
Gamma-glutamyl Trans Peptidase test (GGTP). An increase in GGTP could indicate presence of Primary Biliary Cirrhosis.
57% of patients with acute liver failure have
Anti-transglutaminase Antibodies 3 suggesting a role of
Gluten Sensitivity in primary biliary cirrhosis, and primary biliary cirrhosis is considerable more common in
Gluten Sensitive Enteropathy than the normal population.
45 In some cases of disease protein expression may cause an
Immune Tolerance failure, as might be the case with
Gp210 and
P62 , nuclear pore proteins. Gp210 has increased expression in the bile duct of anti-gp210 positive patients.
6 Both proteins appear to be prognostic of liver failure relative to anti-mitochondrial antibodies.
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 the most frequently used treatment. This helps reduce the cholestasis and improves blood test results (
Liver Function Tests ). It has a minimal effect of symptoms and whether it improves prognosis is controversial. 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 1-56053-499-0. (State of the art; technical.)
- Marshall M. Kaplan, and M. Eric Gershwin, "Primary Biliary Cirrhosis", ''New Engl. J. of Medicine'', 353:1261-1273 September 22, 2005 Number 12 . Review article
- Carlo Selmi, Ross L. Coppel, and M. Eric Gershwin, "Primary Biliary Cirrhosis", in Noel R. Rose, Ian R. Mackey, eds, ''The Autoimmune Diseases'', 4th edition, Academic Press, 2006
- Sanjiv Chopra. ''The Liver Book: A Comprehensive Guide to Diagnosis, Treatment, and Recovery'', Atria, 2002, ISBN 0-7434-0585-4
- 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 1-58333-188-3. her webpage .
- Howard J. Worman. ''The Liver Disorders Sourcebook'', McGraw-Hill, 1999, ISBN 0-7373-0090-6.
