Primary biliary cirrhosis is a rare form of biliary cirrhosis of unknown origin, occurring without obstruction or infection of the major bile ducts, sometimes developing after the administration of such drugs as chlorpromazine and arsenicals. Affecting chiefly middle-aged women, it is characterized by chronic cholestasis (accumulation of bile in the liver) with pruritis, jaundice, and hypercholesterolemia with xanthomas, and malabsorption.

Cirrhosis is a process of advanced scarring of the liver.. The fibrosis or scarring of the liver seen in cirrhosis leads to obstruction of blood flow through the liver. This prevents the liver from performing its critical functions of purifying the blood and nutrients absorbed from the intestines. The end result is liver failure.

Primary biliary cirrhosis, or PBC, is a chronic, or long-term, disease of the liver that slowly destroys the medium-sized bile ducts within the liver. Bile is a digestive liquid that is made in the liver. It travels through the bile ducts to the small intestine, where it helps digest fats and fatty vitamins.

Causes

The cause of inflamed bile ducts within the liver in this condition is not known. The disease more commonly affects middle-aged women. The onset of symptoms is gradual, with fatigue and itching skin as the most common first symptom.

Autoimmune hepatitis. This disease appears to be caused by the immune system attacking the liver and causing inflammation, damage, and eventually scarring and cirrhosis.

Inherited diseases. Alpha-1 antitrypsin deficiency, hemochromatosis, Wilson's disease, galactosemia, and glycogen storage diseases are among the inherited diseases that interfere with the way the liver produces, processes, and stores enzymes, proteins, metals, and other substances the body needs to function properly.

Symptoms

PBC occurs in both men and women, but women get the disease 10 times more often than men. It usually begins between the ages of 30 and 60. Early in the disease, many patients have no symptoms. The only findings may be abnormal blood laboratory results. For example, a high level of the liver enzyme called alkaline phosphatase may be found in the blood. Itching and fatigue are common symptoms later in the disease. Itching is caused by bile entering the bloodstream.

Treatment

Diagnosis is easily made using a blood test for anti-mitochondrial antibody (AMA), which is nearly always positive, combined with the pattern of symptoms.

The current treatment for primary biliary cirrhosis is a bile acid medication, called ursodeoxycholate. Although this medication does not cure the condition, it slows its progression. For advanced staged primary biliary cirrhosis, in which the liver is severely damaged, a liver transplant is the only option.

Liver transplant. When treatments no longer control primary biliary cirrhosis and the liver begins to fail, a liver transplant may help prolong life. Many doctors use a mathematical model to help determine who will benefit from a liver transplant. People with primary biliary cirrhosis who have liver transplants often do very well, although the disease may recur in the new liver.

Some patients have also benefitted from ursodeoxycholic acid (Urso 250 and URSO Forte), which is the only drug approved by the U.S. Food and Drug Administration for the treatment of primary biliary cirrhosis.

The treatment of liver cirrhosis depends upon the root cause of its development. In more severe case one may have to undergo liver transplant.