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.
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.
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.