PRIMARY BILIARY CIRRHOSIS (PBC)

What is Primary Biliary Cirrhosis?
Primary Biliary Cirrhosis (PBC) is a chronic liver disease that slowly destroys the bile ducts in the liver. The bile ducts play an important role in transporting and excreting bile acids, that are formed in the liver, to the intestine. The substance, bile, helps in the digestion of fats in your diet and aids in the excretion of drugs, ions and endogenously produced compounds from the body. Destruction of this duct results in build up of the bile substances in the liver and leads to the destruction of the liver tissue. Continued liver inflammation causes scarring and over time, the disease can progress to cirrhosis.

What causes PBC?
Primary Biliary Cirrhosis is thought to be an autoimmune disease, where the person’s immune system attacks its own tissue, as though the tissues were foreign invaders. The exact cause of the disease is unknown.

In more than 90% of cases, primary biliary cirrhosis affects women between 35 and 60 years of age. It appears to be more common in families, where one member of the family has been previously diagnosed.

What are the signs and symptoms of PBC?
In many cases patients do not show any symptoms of the disease at presentation. They are usually diagnosed through finding an abnormality on routine blood tests or health evaluation.

Early general clinical signs observed in PBC patients vary, and may include fatigue and intense itchy skin (pruritus).

Only in advanced stages, do the main symptoms of PBC start to appear, such as:

Are there other diseases associated with PBC?
Often several other disorders are associated with PBC. The most common is impaired functioning of the tear and salivary glands, causing dry eyes or mouth. Arthritis and thyroid problems may be also present. In the later stages of the disease, bone softening and fragility can occur, leading to fractures and other bone-related complications. Thus some people with PBC may also have osteoporosis, arthritis, and thyroid problems.

Some of the autoimmune diseases that may be associated with PBC include the following:

How is PBC diagnosed?
Primary Biliary Cirrhosis is diagnosed on the basis of several laboratory tests e.g. blood tests, liver function tests and other diagnostic procedures such as liver biopsies. A liver biopsy is a procedure that involves a very small needle to be inserted into the liver, thus enabling the doctor to obtain a tissue sample of the region. The sample is then analysed, and helps in the detection of the extent of liver damage.

The presence of a positive antimitochondrial antibodies (AMA) (blood test) is indicative of PBC in 95% of the cases. Changes that are observed in the patient’s liver function test enzymes are usually the first signs, suggesting that the person has some form of liver impairment or liver disease. Thus, the majority of the PBC diagnosed cases are identified during the asymptomatic stage of the condition.

What is the prognosis?
There is no cure for PBC. The course of PBC is progressive but variable; some patients have minimal symptoms for 10 to 15 years, whereas others deteriorate in only 3 to 7 years. Survival of PBC patients is generally less than that of the general population. If not adequately treated, PBC can lead to death in 5 to 15 years after the diagnosis has been made. There are 4 disease stages of PBC (Stage I to IV; stage IV being the worst). The end stage of PBC is liver failure. Not many of the PBC patients reach the end stage of PBC. In those that do, there is the possible option of liver transplantation.

What are the treatment/management options? Can PBC be treated?
Various medicinal drugs are available to help treat and delay the progression of the disease, as well relieve symptoms such as itchy skin. Preventative treatment may also be given to you, to prevent the development of other symptoms and conditions such as osteoporosis.

Remember, your doctor is the best source of information regarding you and your health. Please consult your doctor if you have any questions about your health, your condition or your medication.

Where can I find more information about this condition or support organisations?
Additional information is available from the following website(s):