What is primary biliary cirrhosis (PBC)?

Primary biliary cirrhosis (PBC) is a form of liver disease that over time can lead to cirrhosis. Much like other forms of liver disease, PBC permanently damages the liver as tissue is replaced with scar tissue (fibrosis). As more scar tissue develops, the structure and function of the liver are affected.

What causes PBC?

The exact cause of PBC is unknown, but it may be related to a problem with the immune system, which is the body's natural defense system.

What are the symptoms?

Many people who have primary biliary cirrhosis do not have symptoms. When symptoms do occur, they may include:

• Itching (pruritus).
• Fatigue.
• Discomfort in the upper right part of the abdomen.

With more advanced liver damage, people may develop complications related to cirrhosis such as:

• Yellowing of the skin and the white part of the eyes (jaundice).
• Fluid buildup in the abdomen (ascites).
• Bleeding of enlarged veins in the esophagus, stomach, and rectum (variceal bleeding).
• Premature thinning of the bones (osteoporosis).

How is PBC diagnosed?

Primary biliary cirrhosis is suspected based on a medical history, physical exam, and lab tests that show high levels of the enzyme alkaline phosphatase and antimitochondrial antibodies (AMA) in the blood. Your doctor may also conduct an imaging test such as a CT scan, which shows images of the liver and other tissues inside the body, and a biopsy, a procedure done to remove a small piece of tissue from the liver. Biopsy is the best way to definitively diagnose PBC.

How is it treated?

Treatment for primary biliary cirrhosis is difficult because the immune system appears to be involved. As with other forms of cirrhosis, treatment focuses on reducing symptoms, preventing and treating the complications of the disease, and preventing other conditions that may cause additional liver damage.

Medicines such as ursodiol may be used to treat itching caused by primary biliary cirrhosis. Ursodiol may also slow the progression of the disease.

Because people with PBC have a high risk of getting osteoporosis, you should have periodic bone mineral density scans. Your doctor may suggest that you take calcium and vitamin D supplements, and perhaps a medicine called a bisphosphonate (such as alendronate or etidronate).

Liver transplantation may be the treatment of choice for people who have end-stage primary biliary cirrhosis, although primary biliary cirrhosis can recur after a liver transplant.