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:

	Jaundice
	Decreased mental status (encephalopathy)
	Ascites
	Gastrointestinal bleeding from esophageal varices
	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:

	Parietal conjunctivitis sicca
	Arthritis
	Scleroderma
	CREST
	Raynaud's Phenomena
	Thyroid disease
	Breast cancer (4 fold increase in risk)
	Renal dysfunction (renal tubular acidosis)

Treatment of primary biliary cirrhosis includes correction of nutritional abnormalities. Patients may develop vitamin deficiencies. Since the deficiencies are usually associated with fat-soluble vitamins, repletion with vitamins A, D, E and K is necessary.

Elevated levels of cholesterol may also be associated with PBC. In fact this is a rather common finding. It is most marked in the early part of the disease.

Medications that lower lipids and cholesterol are appropriate for these individuals. The elevated cholesterol accounts for the fatty deposits noted around the eyes (xanthelasma).

Cholestyramine may be utilized for some of these changes as well.

Ursochenodeoxycholic acid (bile acid therapy) may be extremely useful. In fact, in most cases, this is as effective as a single agent.

Other medications such as aziothioprine (or 6-mercaptopurine), steroids, cyclosporin as well as methotrexate are less effective. In some cases, antifibrotic (compounds which help prevent early scarring of the liver) agents such as colchicine may be used.

The natural history of the disease is one of aggressive development until liver cirrhosis has become established. Many patients do require liver transplantation for their disease.

Complications of PBC

Complications of PBC include:

	Osteopenia
	Hypercholesterolemia
	Fat soluble vitamin deficiencies

Complications of Cholestatic Diseases

Complications of cholestatic diseases include:

	PBC
	Primary sclerosing cholangitis

Both of these disorders are associated with chronic elevations in the liver function tests. This is predominantly characterized by an elevation in the alkaline phosphatase.

Complications of any cholestatic disorder include:

	Osteopenia
	Hypercholesterolemia
	Fat soluble vitamin deficiencies
	Copper accumulation

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