Primary biliary cholangitis (PBC), formerly known as primary biliary cirrhosis, is a chronic liver disease resulting from progressive destruction of the bile ducts in the liver – called the intrahepatic bile ducts.

Bile produced in your liver travels via these ducts to your small intestine where it aids in the digestion of fat and fat-soluble vitamins (A, D, E and K). When the ducts are destroyed, bile builds up in the liver contributing to inflammation and scarring (fibrosis).

Eventually this can lead to cirrhosis and its associated complications, as scar tissue replaces healthy liver tissue and liver function becomes increasingly impaired.

Facts at-a-Glance

1. Primary Biliary Cholangitis affects mostly women, but more men are now being diagnosed.
2. Primary Biliary Cholangitis is a chronic disease in which the small bile ducts in the liver become injured and inflamed and are eventually destroyed.
3. Researchers estimate that in the United States, about 65 out of every 100,000 women have PBC.
4. The disorder usually becomes apparent during middle age, initially affecting most individuals between the ages of 45 to 65 years.
5. There is no cure for Primary Biliary Cholangitis, but there is medication available to manage the disease and slow down the progression of liver damage.

Information for the Newly Diagnosed

What are the signs and symptoms Primary Biliary Cholangitis?

PBC may progress slowly and many people do not have symptoms, particularly in the early stages of the disease. The most common initial symptoms are fatigue and itching of the skin (pruritus). Other symptoms may include:

• Abdominal pain
• Darkening of the skin
• Small yellow or white bumps under the skin (xanthomas) or around the eyes (xanthelasmas)
• Individuals may also complain of having dry mouth and eyes, and bone, muscle and joint pain.
• As the disease progresses, symptoms of cirrhosis can develop including:
• Yellowing of the skin (jaundice)
• Swelling of the legs and feet (edema)
• Enlarged abdomen from fluid accumulation (ascites)
• Internal bleeding in the upper stomach and esophagus from enlarged veins (varices)

Thinning of the bones (osteoporosis) leading to fractures is another complication of PBC. While this is more common in late stages of the disease, it can occur earlier as well. In addition, people with cirrhosis are at increased risk for liver cancer (hepatocellular carcinoma).

What causes Primary Biliary Cholangitis?

The cause of this disease is unknown. It may be related to problems in the immune system. Although PBC is technically not a hereditary disease, meaning a disease caused by a specific gene or genetic defect that is passed from parent to child, there appears to be some family link. PBC is more common among siblings and in families where one member has been affected.

How is Primary Biliary Cholangitis diagnosed?

Because many people with PBC have no symptoms, the disease is often discovered incidentally due to abnormal results on routine liver blood tests. Once PBC is suspected, a blood test to check for antimitochondrial antibody (AMA) is done. This test is positive in nearly all people with PBC. A liver biopsy, where a small sample of liver tissue is removed with a small needle can help confirm the diagnosis. Imaging studies may be used to rule out other diseases, or to further evaluate patients once they have been diagnosed with PBC.

What lifestyle changes may be helpful?

Maintaining a healthy lifestyle can help people with PBC feel better, as well as relieve or prevent some symptoms associated with the disease. Upon diagnosis, your doctor may suggest the following:

• Start a reduced sodium diet if you have fluid overload (edema or ascites)
• Reduce fat intake if you are overweight or have fatty liver
• Drink plenty of water
• Avoid or lower intake of alcohol
• Avoid undue stress when possible
• Start exercising, particularly walking
• Stop smoking
• Maintain good skin care
• Get regular dental examinations

Keep in mind that PBC usually advances slowly over a period of years. Many people lead normal lives for years without symptoms, depending on how early the diagnosis is made. And while there is no cure, people are having good results slowing disease progression and living longer without complications by adhering to their medication regimen and maintaining a healthy lifestyle.

Who is at risk for Primary Biliary Cholangitis?

Women are nine times more likely than men to develop PBC, meaning that women make up about 90% of PBC cases.

The disease most often develops during middle age and is usually diagnosed in people between the ages of 35 to 60 years.

There appears to be a genetic component to developing PBC, as it’s more common among siblings and in families where one member has been affected.

Questions to Ask Your Doctor

• How severe is the liver damage?
• What treatment do you recommend? Will this slow down the progression of the disease?
• Will any medication be prescribed? What are the side effects?
• Should I change my diet?
• Are there any supplements you would suggest that I take?
• What can be done to relieve my symptoms?
• If cirrhosis develops, is transplantation my only option?