Primary biliary cirrhosis

Medical quality assurance by Dr. Albrecht Nonnenmacher, MD at April 5, 2016
StartDiseasesPrimary biliary cirrhosis

Primary biliary cirrhosis is a progressive liver disease that can lead to liver damage. It is an autoimmune disease, but doctors are still not entirely certain of what causes it. However, it is still possible to treat primary biliary cirrhosis and slow its progression.


Definition & Facts

Primary biliary cirrhosis involves the bile ducts of the body being destroyed by inflammation, so bile, which is a digestive liquid made by the liver, remains in the liver instead of traveling to the small intestine.

This buildup of bile damages the liver. Though liver cells can typically regenerate, repeated damage leads to scarring, and cirrhosis occurs. Once cirrhosis happens, the liver cannot function properly and filter out toxins, and this condition can be fatal without treatment. However, primary biliary cirrhosis happens very slowly over several years, so cirrhosis does not happen immediately.

Symptoms & Complaints

At first, there are little to no signs of primary biliary cirrhosis. The first symptom of early stage primary biliary cirrhosis is normally just itchiness of the skin along the legs, back, and arms.

Patients with the condition may also feel constantly fatigued, and they often have dry eyes and a dry mouth. People in the middle stages of primary biliary cirrhosis are also likely to suffer from arthritis, thyroid problems, gallstones, kidney stones, and weakened bones.

Once cirrhosis sets in during late stage primary biliary cirrhosis, symptoms of this condition become more pronounced. Cholesterol levels are elevated, bowel movements may become greasy, and fatty deposits can build up around the eyes, elbows, knees, palms, and soles.

A buildup of fluid can cause the abdomen, feet, or ankles to swell up, and people feel pain in the upper right part of their abdomen. Jaundice may cause the skin and the whites of patients' eyes to become yellow. If left untreated, liver failure] may occur, causing potentially fatal issues such as gastrointestinal bleeding and coma.


Most scientists are still somewhat unclear about what the precise cause of primary biliary cirrhosis is, but it is linked to the immune system. For some unknown reason, some people's immune systems become confused and start attacking the cells of the bile ducts. Though the T lymphocytes created by the immune system are supposed to attack harmful bacteria and viruses, they instead begin to accumulate in the liver and begin to break down the bile ducts completely.

Primary biliary cirrhosis is more common among individuals with a family history of the disease, and genetic factors play a role. It is also more common among white, northern, European females. Certain environmental factors also make a person more likely to develop primary biliary cirrhosis. A medical history of smoking has been strongly linked to the cause of this liver condition, and other toxic chemicals in the environment may cause it.

Many people develop the condition after having an infection of some support, so it is possible that the immune system begins overreacting and attacking the bile ducts after it has been fighting off a bacterial infection.

Diagnosis & Tests

Since there are so few symptoms at first, it is very difficult to diagnose early stages of primary biliary cirrhosis based on just a medical examination. Sometimes, it is not even noticed until the liver is already failing, but in many cases, routine blood tests make it clear that the liver is not functioning properly.

Once primary biliary cirrhosis is suspected, doctors can conduct a few different tests in order to properly diagnose it. A blood analysis can show the presences of anti-mitochondrial antibodies, which are very rare in people who do not have primary biliary cirrhosis.

Imaging tests, including ultrasounds, MRIs, and X-rays can all be used to examine the liver and bile ducts to see if they are functioning properly. If the doctors are still not positive that a patient has primary biliary cirrhosis after doing blood tests and imaging tests, they can perform a liver biopsy, which will tell them whether or not the patient has the disease.

Treatment & Therapy

There is no way to entirely cure primary biliary cirrhosis, but fortunately, there are many ways to slow the progression of the disease and reduce symptoms of the disease. The most common treatment for this condition is ursodeoxycholic acid, which is sold under the brand names of Actigall® and Urso®. If primary biliary cirrhosis is noticed during the early stages, taking ursodeoxycholic acid can greatly reduce the risk of liver damage and prolong life.

Some people do not respond to ursodeoxycholic acid immediately, but they may start to improve if fenofibrate or bezafibrate, which are both antiviral drugs, are added to their treatment plan. If the condition was diagnosed at a later stage and liver failure is already occurring, a liver transplant may be required. There are some experimental drugs that may be able to treat the condition by suppressing the immune system response. These drugs include methotrexate and colchicine.

Prevention & Prophylaxis

Because so little is known about primary biliary cirrhosis, it is difficult to determine the best method of preventing the condition. In general, avoiding smoking and other environmental toxins reduces the risk of getting the disease. For people who have a family history of people with the liver condition, regular blood tests can help doctors to diagnose and treat the primary biliary cirrhosis before liver damage occurs. Living an overall healthy lifestyle that does not damage the liver with alcohol can also help to lessen the chance of liver problems.