Primary Biliary Cirrhosis

What is Primary Biliary Cirrhosis?

Primary Biliary Cirrhosis is the old name for primary biliary cholangitis or PBC. However, many doctors and medical websites still use the old name. The name was changed because liver damage or cirrhosis does not occur until the very last stages of the disease. When treated early enough, liver damage does not occur except at the bile ducts. PBC is a disease that destroys the liver bile ducts, tube-like structures that connect the liver to the small intestine.

Bile ducts transport yellowish bile so that vitamins A, D, E and K are properly absorbed into the body. Bile is not meant to stay a long time in the liver. When it does, problems start. The main cause of PBC is unknown, although genetics, smoking and exposure to toxins may play a part.

What are the Symptoms of Primary Biliary Cirrhosis?

The first symptoms of PBC are feeling tired all of the time; skin suddenly becomes itchy; itchy patches of skin darkens; dry eyes and dry mouth.

Other common symptoms include abdominal pain, muscle pain, joint pain, small white or yellow bumps appearing around the eyes or under the skin, swelling in the feet and legs, swollen abdomen from fluid retention, yellowing of the whites of the eyes, yellowing of the skin, passing blood due to bleeding in the intestines; development of osteoporosis or brittle bones.

Primary Biliary Cirrhosis Causes

Primary Biliary Cirrhosis is now generally referred to as Primary Biliary Cholangitis or PBC. The condition occurs when the bile ducts in the liver slowly deteriorate causing toxic substances, old red blood cells and cholesterol to build up in the liver instead of being processed out. This can lead to permanent damage and scarring of the liver over time and ultimately to liver failure. Roughly 90% of all patients with PBC are women between the ages of 40 and 60. The actual cause of PBC is unknown. It is suspected to be an autoimmune disease that is potentially triggered by some sort of bacterial infection or other environmental factor(s). There does, however, seem to be some behaviors and conditions that put the patient at higher risk for developing PBC. The first are factors like gender, age and heredity. PBC does seem to occur in families, although the actual gene is unknown at this time. Other factors are smoking, exposure to toxic chemicals and bacterial infections.

How is Primary Biliary Cirrhosis Treated?

There is no cure for PBC, although the symptoms can be treated and further damage to the liver can be stopped with treatment. Medications used include ursodiol to help remove bile from the body and obeticholic acid helps the body not make so much bile.

The patient also needs to make lifestyle changes to diet, exercise and stress management in order for the medicines to work their best. Vitamin and calcium supplements may be recommended or prescribed.

People with any sort of liver damage or with PBC should never eat raw shellfish because the shellfish may cause a potentially lethal infection.

Only in the worst late stage of the disease is a liver transplant the only option a patient has to stay alive.

Primary Biliary Cirrhosis Prevention

The last three factors are things that can be controlled or prevented altogether. Smoking is directly associated with the development of Primary Biliary Cholangitis. Avoidance of smoking, or cessation of smoking, and limiting exposure to cigarettes and second hand smoke would be a preventive measure. Limiting exposure to toxic chemicals, especially if other members of the patient’s family have had PBC, could also be a critical way to avoid the onset of PBC. Finally, good hygiene and smart management of one’s health can be a preventive measure, again particularly if the genetic tendency toward PBC is already present. Simple behaviors like washing hands and avoiding people with infections could help avoid development of PBC. A healthy diet that limits cholesterol and fats, alcohol intake and sugar is a good way to maintain overall health of the liver which helps prevent PBC as well.

Resources
Last Reviewed:
October 09, 2016
Last Updated:
January 11, 2018