Primary Biliary Cirrhosis

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PRIMARY BILIARY CIRRHOSIS

Primary Biliary Cirrhosis is a slow-acting, chronic liver disease, which causes progressive destruction of the microscopic bile ducts in the liver. The body's own defence mechanisms attack the cells lining the bile ducts within the liver as if they are foreign invaders.

The bile ducts are the channels through which bile acids normally used in digestion flow. The resulting damage impairs the drainage of bile, and the build up of bile acids causes inflammation and scarring which may after many years become extensive.

This replacement of liver cells by fibrous tissue is called cirrhosis, and it may eventually obstruct the flow of blood through the liver so that it can no longer function normally. However, many people who have PBC never progress to the stage of cirrhosis.

FUNCTIONS OF THE LIVER

Converts food into chemicals necessary for life and growth
Produces quick energy when it is needed
Manufactures new body proteins
Prevents shortages in body fuel by storing sugars, vitamins and minerals
Aids the digestive process by producing bile
Digests fat
Remove toxic substances from the blood
Controls the production of cholesterol
Maintains hormone balance
Stores iron
Produces factors essential to blood clotting
Produces immune factors to help the body resist infection
Regenerates its own tissue

The liver is a very uncomplaining organ and has the capacity to carry on its many functions with only a small portion in working order.

WHAT CAUSES PBC?

At present the cause of PBC is unknown, but it is thought to be an autoimmune disease - the body's immune system malfunctions and turns on itself. PBC is not contagious, and is not diet or alcohol related. It seems to be more common in families where one member of the family has been previously affected - probably about one patient in thirty to seventy has a close relative with the disease.

Research has indicated that people with PBC display several abnormalities of the immune system, but what initially sparks off the bile duct damage is unknown. Generally PBC presents in women between thirty and fifty-five but it can also present in younger and older women and sometimes in men. About one in every ten cases are men.

SYMPTOMS OF PBC

Some PBC sufferers are asymptomatic ie. they display no outward symptoms of PBC. Others may have one or more of the following:

Chronic fatigue and lethargy - mild or severe
Intense itching of the skin which may affect any part of the body
Frequent indigestion or nausea, poor appetite
Small yellow or white bumps under the skin
Sore joints/bone pain
Easy bruising or bleeding
Dry eyes and/or mouth
Persistent ache or discomfort in the upper right hand side of the abdomen
Dark urine and/or pale stools
Darkening of the skin resembling a tan
Osteoporosis
Weakness in wrists and/or hands

As the disease progresses other symptoms may be present:

Elevated liver test enzymes
Jaundice - yellowing of the skin or eyes
Enlarged abdomen due to fluid accumulation (ascites)
Hepatic encephalopathy causing personality changes,dulling of mental functions, confusion and forgetfulness

Those with PBC often look extremely healthy. The slight pigmentation of the skin that is often present, together with a tinge of jaundice, often makes the individual look tanned and fit. Unfortunately these outward appearances often mask what is going on inside.

RELATED CONDITIONS

There are sometimes other autoimmune diseases associated with PBC such as:
Sjogren's syndrome
Rheumatoid Arthritis
Raynaud's disease
Scleroderma
Thydroiditis
Pancreatitis

DIAGNOSIS OF PBC

The presence of liver disease is detected by liver function tests from a blood sample. People with PBC nearly always have a specific antibody, AMA (antimitochondrial antibody) and finding this in the blood is helpful in reaching a diagnosis. This can usually be confirmed by biopsy, which involves taking a very small sample of cells from the liver with a needle. A biopsy also helps to determine the extent of any damage.

TREATMENT OF PBC

Although there is presently no cure for PBC, ursodeoxycholic acid (Ursofalk) has been shown in many trials to influence long term prognosis and life expectancy. In many people Ursofalk also improves symptoms such as itching, nausea and fatigue.

Other medications your doctor may may prescribe to help ease the itching are cholestyramine and rifampicin.

Measures can be taken to reduce bone loss including weight bearing exercise and increasing calcium intake. Some women may benefit from hormone replacement therapy and this should be discussed with your doctor.

Like most chronic liver diseases, PBC can impair the capacity of the liver to break down toxic drugs and chemicals. These include prescribed and over the counter medicine, alcohol and even foods.

As the digestion and absorption of foods may also be impaired, a balanced diet should be eaten, rich in calcium, vitamins and proteins.

PROGNOSIS

PBC may take a long time to develop and the severity varies greatly from one patient to another. Many have few or no symptoms for many years and some live with a benign form of the illness with little or no discomfort. Many do have symptoms but never reach end stage of PBC.

For those that do reach this stage liver transplantation may be an option, especially where the quality of life is deteriorating. Current results for liver transplant in PBC are excellent.