Primary biliary cirrhosis (PBC) is a long-term autoimmune liver disease in which the bile ducts in the liver become damaged, which gradually leads to a build-up of harmful substances in the liver that can damage it and eventually lead to irreversible scarring of the tissue.
Although some people with primary biliary cirrhosis remain symptom-free for years after they're diagnosed, others experience a number of signs and symptoms. Common early symptoms include fatigue, itchy skin and dry eyes and mouth. Later symptoms may include:
- Pain in the upper right portion of the abdomen;
- Bone, muscle or joint pain;
- Yellowing of the skin and eyes;
- Darkening of the skin that is not related to sun exposure;
- Swollen feet and ankles;
- Buildup of fluid in the abdomen due to liver failure;
- Fatty deposits on the skin around the eyes, the eyelids, or in the creases in the palms, soles, elbows or knees;
- Weak and brittle bones;
- Elevated cholesterol;
- Diarrhea that can be greasy;
- Underactive thyroid
The exact cause of PBC is unknown, although it is widely thought to be the result of a problem with the immune system. Most experts consider primary biliary cirrhosis an autoimmune disease in which the body turns against its own cells. In such cases, the immune system sends specialised cells that usually kill bacteria and viruses to the bile ducts. They gradually become scarred and disrupt the flow of bile out of the liver. This damage can gradually cause the liver to lose its function, which is potentially fatal.
Others experts believe that it is triggered by a combination of genetic and environmental factors.
The following categories are considered to be at greater risk of suffering from PBC:
- People at the age between 30 and 60 years.
- People whose family members have had the disease
Researchers think that genetic factors, when combined with environmental factors, trigger primary biliary cirrhosis. These environmental factors may include infection, smoking and environmental toxins.
The complications of primary biliary cirrhosis develop as the ability of the liver to function decreases. As liver damage progresses, people with primary biliary cirrhosis may develop a number of complications and serious problems, including:
- Portal hypertension;
- Enlarged spleen;
- Easy bruising or bleeding;
- Gallstones and bile duct stones;
- Enlarged veins;
- Liver cancer;
- Vitamins A, D, E and K deficiencies;
- Decreased mental function.
Since the cause of the disease is not known, it is not possible to prevent PBC. However, medications may be able to slow the progression of the disease and control the symptoms. Should a person develop PBC, all attempts should be made to prevent further potential damage. This may include limiting alcohol intake, avoiding the use of medications that contain acetaminophen and cholesterol lowering agents.