Primary biliary cirrhosis

August 13th, 2012 | Posted by admin in Diseases and Conditions

Primary biliary cirrhosis (PBC) is a disease that slowly damages the bile ducts in the liver. Over time, the bile flow is restricted and the bile builds up in the liver. This damages the liver cells, which in some cases leads to cirrhosis (scarring of the liver). Common symptoms are itching and fatigue early. You have other symptoms as the disease progresses. Drugs aimed at relieving symptoms and slow progression of the disease can be used. A liver transplant may be needed if the liver is damaged.

What does the liver?
What are bladder and bile ducts?
What is primary biliary cirrhosis?
What causes primary biliary cirrhosis?
Who gets primary biliary cirrhosis?
What are the symptoms of primary biliary cirrhosis?
How is primary biliary cirrhosis diagnosed?
What is the treatment for primary biliary cirrhosis?
Do I need to take other precautions?
What is the outlook (prognosis)?
More information and advice
References

What does the liver?

liver

The liver in the right upper abdomen. Its features include:

Glycogen storage (fuel for the body) made from sugars. When required, the glycogen is broken down into glucose that is released into the bloodstream.
Helping to process fats and proteins from digested food.
The production of proteins that are essential for blood clotting (coagulation factors).
Processing many medicines you can take.
Helping to remove or process alcohol, poisons and toxins from the body.
Making bile which passes from the liver to the intestine to help digest fats.

What are bladder and bile ducts?

Detail of the upper abdomen showing the bile ducts

Bile is a yellow-colored liquid containing green various chemicals and bile salts. Bile helps digest food, especially fatty foods. It also helps the body absorb certain vitamins (A, D, E and K) of the foods you eat.

Bile is made by the liver cells. Faint liver cells in small tubes called bile ducts. A network of bile ducts in the liver. They join together (like the branches of a tree) to form the common bile duct larger. Bile constantly drips from the small bile ducts, common bile duct, and duodenum (part of the intestine – the first part of the small intestine).

The gallbladder is located under the liver. It's like a bag out of the common bile duct and is filled with bile. It's like a reservoir that stores bile. The gallbladder squeezes (contracts) when we eat. This empties bile stored in the back of the common bile duct and into the duodenum.

What is primary biliary cirrhosis?

PBC is a condition that affects the bile ducts. It's called:

Elementary – because the cause is not known. (Ie, not secondary to any known cause, such as alcohol or poisons.)
Biliary – because it affects the bile ducts.
Cirrhosis – because as the disease progresses can damage liver cells and cause scarring in the liver. Cirrhosis means scarring of the liver and has many causes. PBC is a rare cause of cirrhosis.

In PBC, inflammation develops around the small bile ducts within the liver. Slowly inflammation can damage the ducts. Bile ducts are obstructed due to inflammation gradually. Bile flow is not capable of any type of downward conduit blocking. This process tends to get worse very slowly, and the number of damaged and blocked bile ducts is gradually increased. If bile can not flow through the bile ducts, bile accumulates in the liver cells. Over time, the liver cells is also damaged by the accumulation of bile. Bile also can spread to the blood.

As the liver cell damage becomes worse, scarring (cirrhosis) may gradually develop in the liver. Over time, cirrhosis can cause liver failure and other serious problems.

In the early stages of the disease, the main problem is the accumulation of substances in the liver and in the blood normally flows into the duodenum, as part of bile. Cirrhosis occurs only in the later stages of the disease. The rate of decline in the early stages of the disease later, more severe stages of the disease can vary from person to person.

In fact, the term PBC is controversial because not all people with PBC develop cirrhosis. Moreover, if cirrhosis occurs, usually develops over several years after the first disease begins (after several years of inflammation of the bile ducts).

What causes primary biliary cirrhosis?

The exact cause is not known. It is believed that the PBC is an autoimmune disease. Normally, the immune system attacks bacteria, viruses and other germs with antibodies, white blood cells, etc. in individuals with autoimmune diseases, the immune system attacks the body's tissues. It is not clear why this happens. Some people seem to have a tendency to develop autoimmune diseases. In these people, which could cause the immune system to attack the body's own tissues. The exact trigger is unknown, but potential triggers that have been suggested are some kind of infection or some type of toxin (poison). There may also be a genetic tendency for PBC, as it seems to run in some families.

In people with PBC, the immune system attacks the cells lining the small bile ducts in the liver. This causes damage and inflammation in and around the bile ducts in the liver.

People with primary biliary cirrhosis have a higher risk of developing other autoimmune diseases. For example, Sjogren's syndrome, thyroid disease, Raynaud's phenomenon and scleroderma.

Who gets primary biliary cirrhosis?

PBC is a rare condition. It affects 1 in 5,000 people in the UK. About 9 out of 10 cases occur in women. But usually develops in women between 30 and 65.

What are the symptoms of primary biliary cirrhosis?

The most common early symptoms include:

Tiredness (fatigue) is often the first symptom to develop. It can be a good type of disabling fatigue. The reason PBC fatigue causes are unclear.
Itching (pruritus) is a common symptom, but not always. Sometimes it's serious and painful, with itching in the whole skin. The cause of itching is unclear. It may be due to a chemical of bile that accumulates in the bloodstream.
Discomfort in the liver (right upper abdomen) occurs in about 1 in 5 cases.
Red spots or pink hands can develop.
Some people develop nausea (feeling sick), bloating or diarrhea. Stool (feces) may be pale, bulky and difficult to pass away (called steatorrhea or fatty stools), due to the difficulty of your body has to digest fat when you have PBC.

When a doctor examines you, he or she may feel that your liver is enlarged. As the disease progresses, jaundice (yellowing of the skin). This is due to an accumulation of bilirubin (a bile chemical) in the bloodstream. If cirrhosis develops, other liver-related symptoms are likely to develop.

Some people have no symptoms with PBC

PBC is sometimes diagnosed by chance when blood tests that are performed for other reasons show anomalies due to PBC. Some of these people never develop symptoms. However, many of these people have symptoms at some point, as the disease progresses.

Symptoms related disorders

As mentioned, some people with PBC have other autoimmune diseases. Therefore, the symptoms associated with these diseases also occur in some cases. For example, dry mouth and dry eyes are the main symptoms of Sjogren's syndrome.

Symptoms of complications

The risk of developing osteoporosis is greater in women with PBC. (See separate leaflet called 'Osteoporosis' for more details.) Other complications associated with liver function disorders may develop in some cases. For example, problems with the kidneys and pancreas. Liver cancer is also a rare complication.

How is primary biliary cirrhosis diagnosed?

Blood tests

If PBC is suspected symptoms, a blood test usually confirms the diagnosis. Most people with PBC have:

High levels of certain liver enzymes (chemicals) in the bloodstream. (See separate leaflet called 'Blood – liver function tests. For details)
An antibody called anti-mitochondrial antibodies. This antibody may have something to do with the cause of the disease, since it attacks the cells called mitochondria.

Many people with PBC also have a high cholesterol level. However, this is usually mainly the good high density lipoprotein (HDL), which means that there is increased risk of heart disease. (See the booklet called 'cholesterol' for more information.)

Ultrasound scan

Your doctor can arrange for you to have an ultrasound of the liver. Gel is applied to the skin of your abdomen and the ultrasound probe is moved across the skin (similar to exploration that women have during pregnancy). This allows the doctor to bile ducts and check that their symptoms are not caused by other conditions.

Liver biopsy

Liver biopsy is when a small sample of tissue is removed from a part of the liver. The sample is examined under a microscope to check for abnormal cells. Typical changes observed under the microscope of the biopsy sample can confirm the diagnosis of PBC. The biopsy may also give an indication of severity. For example, cirrhosis is present, and if so, how bad. (See separate leaflet called 'Biopsy – Liver. For details)

What is the treatment for primary biliary cirrhosis?

There is no cure for PBC. The goal of treatment to relieve symptoms and to halt the progression of the disease. A liver transplant can save the life in the later stages of the disease.

Treatment to relieve symptoms

Itching can be a troubling symptom, and can be difficult to treat. Antihistamines can be used initially to help relieve itching. However, cholestyramine (Questran ®) is the most commonly used medication for itching. It works by binding to bile in the intestine. (When bile enters the intestine, which moves toward the large intestine, where a portion is absorbed in the bloodstream, and goes back to the liver for reuse. As cholestyramine binds the bile in the large intestine , to stop this reabsorption and bile so more than usual is eliminated in the feces (stool). This helps to reduce the accumulation of bile in the liver and blood, which often relieves itching.) can having a delay of 1-4 days after treatment before improvement itching. Other binding bile medicines are sometimes used.

Other drugs that are sometimes used to relieve itching if the above are not helpful. For example, rifampicin. It is unclear how, but they help in some people. Ursodeoxycholic acid (see below) can also relieve itching. Plasmapheresis (plasma exchange like) has been used in some cases of severe and persistent itching.

Dry skin can make itching worse, so using a generous amount of moisturizer is useful if you have dry skin.

No drugs appear to alleviate the fatigue that often is a major symptom.

The treatment to slow progression of the disease

There is no medicine to stop or reverse the disease. However, some medications can slow the progression of the disease in some patients.

Ursodeoxycholic acid (UDCA, or URSO) is the most common drug used in order to slow the progression of the disease. It works by changing the composition of bile in the liver which can reduce the damaging effect of bile in the liver cells. However, its effectiveness is controversial and may not work on everyone. Some studies have shown that seems to work better in the early stages of PBC. Some evidence suggests that UDCA may help delay the need for a liver transplant in some patients with PBC and can help keep you alive longer. However, its effect on the later stages of PBC (in people who have developed cirrhosis) does not seem as good. It can also help relieve itching.

Immunosuppressive drugs are sometimes used. For example, penicillamine, azathioprine, methotrexate, cyclosporin and steroids. These work by suppressing the immune system. But again, there is little clear evidence to say how effective they are in the treatment of PBC. They all have a risk of causing significant side effects.

Your specialist will advise you on the pros and cons of trying any particular drug.

Treatment of diseases associated

As mentioned, various autoimmune diseases are more common in people with PBC. Therefore, you may need treatment for these too.

Osteoporosis is more common in women with PBC. The prevention and treatment of osteoporosis is the same as for any other woman and is described in a separate booklet called "osteoporosis".

Treatment of cirrhosis and liver failure

See separate leaflet called 'Cirrhosis' for details about cirrhosis. Liver transplantation is an option if the liver is severely damaged due to cirrhosis. This is a major operation and is not due to the light. However, it can be a life-saving measure and the results are often very good. Some people with severe itching that has not responded to any other treatment for a liver transplant, even if your liver is not badly damaged.

Sometimes, PBC can come back and haunt the transplanted liver. However, this can not happen and if it does, it can take up to 15 years before it returns.

Do I need to take other precautions?

Alcohol

If you have PBC, you may no longer able to deal with alcohol. Some people just drink a small amount of alcohol on special occasions. The amount of alcohol that is reasonable for you to take may vary from person to person and depends on the degree of damage to your liver. You should ask your doctor who will advise you on whether you can drink alcohol and how much alcohol is reasonable for you.

Medication

You should always remember to tell a doctor or a pharmacist who has PBC before starting any medication (including drugs, supplements or medicines you can buy without a prescription). This is because a lot of drugs are processed in the liver. Because your liver is not working so well if you have PBC, you can get some unwanted effects of certain medications.

What is the outlook (prognosis)?

PBC is a progressive disease. However, in many cases, the disease progresses very slowly over a number of years. Many people may have very few problems for perhaps decades. At the onset of the disease is difficult to predict if and when the disease will progress to cirrhosis and liver failure. Addressing the common symptoms of ringworm and fatigue can be difficult. Research continues to try to identify drugs that may be more effective in preventing disease progression than those currently available.

More information and advice