Primary Biliary Cholangitis (PBC)

Fact: Women are nine times more likely than men to develop PBC

What is Primary Biliary Cholangitis?
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.

What are the signs and symptoms of PBC?
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 PBC?
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 It Diagnosed?
If you have any of these symptoms, your doctor might suggest blood tests. They’ll tell the doctor how well your liver works and if you have any immune system issues. The doctor may also want you to have imaging tests like an ultrasound, CT scan, MRI, or X-rays. These provide a more detailed look at your liver and the area around it. The doctor may also suggest a liver biopsy. They’ll remove a small piece of liver tissue and examine it in the lab to look for damage or disease.

Treating the Disease
There is no cure for PBC, however, there are medications that can help slow disease progression and manage symptoms. Ursodiol (brand names Actigall, URSO 250, URSO Forte) is a naturally occurring bile acid (ursodeoxycholic acid or UDCA) that helps move bile out of the liver and into the small intestine. If used early enough, Ursodiol can improve liver function and may keep you from needing, or delay the need for a liver transplant. People with PBC must take this medication every day for life.

UDCA is effective in more than 50 percent of patients, but up to 40 percent of patients do not achieve an adequate reduction in alkaline phosphotase (ALP) or bilirubin with UDCA, while 5-10 percent are unable to tolerate UDCA.

Treating the Symptoms
• Intense itching is one of the most common symptoms of PBC. Overthe- counter antihistamines like diphenhydramine (Benadryl) may be helpful. Other agents such as rifampicin, naltrexone, cholestyramine and sertraline may be prescribed.
• Dry eyes can be relieved by using eye drops (artificial tears).
• A dry mouth may be helped by sucking on hard candy or chewing gum, both of which increases saliva. There are also saliva substitutes and some medications that can be used.

Preventing Complications
• Blood tests to monitor for deficiencies in fat-soluble vitamins are often done. As PBC progresses, some people need to replace the fat-soluble vitamins lost in fatty stools, so you may be put on vitamin A, D, E and K replacement therapy.
• Since people with PBC are at a higher risk for osteoporosis, calcium and vitamin D are usually prescribed. A baseline bone density is currently recommended at the time of initial diagnosis.
• As the ability of the liver to function decreases over time, complications associated with cirrhosis will need to be addressed and treated. Screening for varices and liver cancer is often recommended.

What lifestyle changes may be helpful for PBC patients?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 alcoho
• Avoid undue stress when possible
• Start exercising, particularly walking
• Stop smoking
• Maintain good skin care
• Get regular dental examinations

Who is at risk for PBC?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

Information on this page taken directly from:
American Liver Foundation

Want to learn more?
The following are organizations and/or websites dedicated to providing information and education surrounding 
Primary Biliary Cholangitis (PBC). These organizations are dedicated to research, education, awareness, and/or support. They are listed in Alphabetical order without any preference or prejudice. Listing these organizations is not a recommendation or referral in any regard for seeking treatment or consultation or support for treatment.

American Liver Foundation
Cleveland Clinic