Primary Biliary Cirrhosis (PBC)

Primary Biliary Cirrhosis (PBC)

Dr. KennedyPrimary biliary cirrhosis (PBC) is a chronic (what is believed to be autoimmune) disease which affects primarily the bile ducts of the liver. PBC also may be called "chronic nonsuppurative destructive cholangitis" or "primary autoimmune cholangitis." Primary biliary cirrhosis may affect up to 1 in 4,000 people (although some sources report 1 in 10,000) and the sex ratio is 10 to 1, women to men.

Autoimmune disorders are caused when the body’s immune system, which is meant to defend the body against bacteria, viruses, and any other foreign product, malfunctions and produces antibodies against healthy tissue, cells and organs. Why this happens is unknown, but it may be that the immune system is reacting to a foreign organism or toxin and the antibodies it produces also atttacks the hosts tissues.

PBC is characterized by inflammatory destruction of the system of ducts in the liver which drain bile into the small intestines. Chronic inflammation causes scar tissue to form in the bile ducts and results in interference with the excretion of bile. The following signs and symptoms may be present in PBC: fatigue, pruritus (itchy skin), jaundice (yellowing of the eyes and skin due to increased bilirubin in the blood), xanthelasmata (focal collections of cholesterol in the skin, especially around the eyes). Complications of cirrhosis and portal hypertension include fluid retention in the abdomen (ascites), esophageal varices (dilated veins in the esophagus), hepatic encephalopathy even causing coma in extreme cases. There is an association with an extrahepatic autoimmune disorders Rheumatoid Arthritis and Sjögren syndrome in up to 80% of cases.

It is now recognized that there are antibodies manufactured which destroy the cellular energy factories (mitochondria) of the bile duct system of the liver. Cirrhosis of the liver is the breakdown of the normal cellular architecture of the liver with scarring and is a late manifestation of the disease. PBC ranges from very mild, when it may not even be detected, to a life-threatening illness. Early diagnosis and treatment is important to the outcome of the illness. Conventional treatment involves the use of Prednisone and other corticosteroids. Often patients are unable to tolerate the toxic side effects of these drugs.

PBC must be distinguished from other conditions with similar symptoms, e.g. autoimmune hepatitis and primary sclerosing cholangitis (PSC). Diagnostic blood tests include: liver function tests (high alkaline phosphatase, elevated AST, ALT), antimitochondrial antibody, antinuclear antibody. M2-IgG antimitochondrial antibody is the most specific test. Now most patients are diagnosed without invasive investigation since the combination of anti-mitochondrial antibodies and typical (cholestatic) liver function tests are considered diagnostic. Anti-nuclear antibodies appear to be prognostic of where the disease is headed 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. Anti-np62 and anti-sp100 are also found in association with PBC. However, a liver biopsy is necessary to determine the stage of disease.

Mainstream medical treatment has little to offer patients with PBC until the end-stage and then liver transplant can be a life saving procedure. However, in alternative medicine we have great success with the use of intravenous lipoic acid therapy.

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