Primary biliary cirrhosis (PBC) is a chronic disease of the liver. It affects the small, microscopic, bile ducts within the liver. These are characterized by destruction of these bile ducts.
In most cases the disease is unknown until it develops cirrhosis with all of the attending complications from cirrhosis of the liver.
The disease is more frequently recognized now that blood serology for anti-mitochondrial antibody (AMA) and liver function tests can be performed.
This disease affects women more than it does men. In fact, it is approximately 9-10 times more common in women then men.
With this disease, the alkaline phosphatase is elevated. When this is seen, a subsequent test entitled an anti-mitochondrial antibody can be sent for confirmation of the disease. In 95 percent of cases, anti-mitochondrial antibody (AMA) will be elevated. The other 5 percent of patients do not show this feature. Under those circumstances, a liver biopsy is performed in order to confirm or eliminate this possible diagnosis.
The incidence of primary biliary cirrhosis differs according to different studies. This may be secondary to reporting differences in different nations of the world.
The prevalence of this disease is thought to be between 100 and 150 million cases worldwide.
Clinically, patients are usually asymptomatic (without symptoms or physical complaints). As the disease progresses, patients develop symptoms. These are usually fatigue, itch and malaise. Further into the course of PBC, malabsorption of fat-soluble vitamins may occur. This can result in deficiencies of vitamin A, D, E and K.
When primary biliary cirrhosis has developed into advanced cirrhosis, patients develop complications associated with cirrhosis of the liver including:
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Jaundice |
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Decreased mental status (encephalopathy) |
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Ascites |
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Gastrointestinal bleeding from esophageal varices |
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Portal gastropathy |
In general the most common symptoms associated with PBC is fatigue. This occurs in approximately 70 percent of cases. Pruritis (itching) is associated in approximately 65-70 percent of cases. As many as 30 percent of cases may have no features at all. Other features which are significant are hyperpigmentation of the skin (55 percent), enlarged liver (50 percent), enlarged spleen (30 percent) and fatty deposits around the eyes (xanthelasma) which occurs in perhaps 20 percent of the cases. Diseases associated with PBC include:
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Parietal conjunctivitis sicca |
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Arthritis |
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Scleroderma |
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CREST |
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Raynaud's Phenomena |
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Thyroid disease |
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Breast cancer (4 fold increase in risk) |
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Renal dysfunction (renal tubular acidosis) |
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Other Liver Pages:
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Albumin |
Alcoholic Cirrhosis |
Alcoholic Liver Disease |
Alpha 1 Antitrysin Deficiency |
Autoimmune Hepatitis |
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Common Bile Duct Stones |
Esophageal Varices |
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Iron Overload Diseases |
Primary Biliary Cholangitis |
Primary Sclerosing Cholangitis |
Wilson's Disease
Related Links
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Liver Function Tests
Patient Education:
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