Primary Biliary Cholangitis (PBC)

What is primary biliary cholangitis?

Primary biliary cholangitis (PBC) is a long-term autoimmune disease that slowly damages the bile ducts inside your liver. Over time, this damage can cause liver tissue scarring and lead to serious liver disease and liver failure if the disease progresses and you do not receive treatment.

Your bile ducts normally carry bile from your liver to your small intestine. Bile is a fluid that helps you digest fats and absorb some vitamins, With PBC, your immune system mistakenly attacks the lining of the small bile ducts inside your liver. 

These ducts can become inflamed and injured and then bile can’t flow out of the liver the way it should. Bile builds in the liver, causing inflammation and scarring (fibrosis). Over the years, this buildup can damage your liver cells and lead to severe scarring (cirrhosis). Sometimes, PBC can cause liver failure.

PBC used to be called primary biliary cirrhosis but the name changed because many people are now diagnosed and treated before they develop cirrhosis.

What causes PBC?

Experts do not know exactly what causes PBC. They do know it is an autoimmune disease, which means your immune system attacks healthy cells in your body as if they were germs. 

These factors may play a role:

• Genetics: PBC tends to run in families.
• Immune system changes: People with PBC often have other autoimmune conditions, such as thyroid disease or Sjögren’s syndrome.
• Environment: Triggers may include urinary or gut infections, cigarette smoking and exposure to certain chemicals or pollutants.

PBC is not caused by drinking alcohol or by a viral hepatitis infection. 

Who is at risk?

PBC can affect anyone but it is much more common in certain groups:

• Most people with PBC are women
• Most people are diagnosed between ages 35 and 65
• Having a close relative with PBC slightly raises your risk
• Having another autoimmune disease increases your risk

PBC is seen around the world but appears more often in North America and Northern Europe. It is considered an uncommon disease but providers are diagnosing it more often. That’s because awareness and testing methods are improving.

Symptoms of primary biliary cholangitis

PBC usually develops slowly. Many people with it feel fine at first and learn they have PBC when routine blood tests show changes in liver function.

Early or mild symptoms

Common early symptoms include:

• Feeling very tired, even after you rest
• Itchy skin
• Dry eyes 
• Dry mouth
• Dry or sensitive skin
• Trouble with memory, focus or “brain fog”

These symptoms can be mild to severe. They do not always match the stage of the disease.

Later symptoms

As the disease progresses, you may notice:

• Yellowing of the skin or eyes (jaundice)
• Pain or discomfort in the upper right side of your abdomen
• Swelling in your legs or belly
• Easy bruising or bleeding
• Darkening of the skin
• Small yellow or white fatty deposits on or under the skin, especially around the eyes or in skin folds
• Oily, pale or greasy stools and diarrhea from trouble digesting fats
• Unintended weight loss or poor appetite

Signs of advanced disease

In more advanced stages, bile and toxins can build up and blood flow through the liver can change. This may lead to:

• Jaundice that gets worse
• Excess fluid in the belly or legs
• Bleeding veins in the esophagus or stomach 
• Confusion, sleepiness or trouble thinking clearly

These are warning signs that you need urgent medical care.

How PBC is diagnosed

In many cases, your provider may think you might have PBC after a routine blood test shows abnormal liver enzymes and changes in your liver function. They will ask about your symptoms and medical history and examine you. They will rule out other conditions, including primary sclerosing cholangitis (PSC).

Blood tests

Key blood tests to diagnose primary biliary cholangitis include:

• Liver enzymes that show a cholestatic pattern, especially high alkaline phosphatase (ALP) levels
• Tests for specific antibodies linked to PBC, most often antimitochondrial antibody (AMA)

Imaging and other tests

Your provider may also order:

• Ultrasound, CT scan or MRI to look at your liver and bile ducts and to rule out a blockage
• A liver biopsy to confirm the diagnosis or to better understand how advanced the disease is

Finding PBC early gives you more options to slow the disease and prevent complications.

Treatment for primary biliary cholangitis

There is no cure for PBC but treatment can slow the damage to your liver, relieve your symptoms and help you live longer and better.

Medicines that target the disease

The main treatments include:

• Ursodeoxycholic acid (also called ursodiol): This medicine helps move bile out of the liver. It can slow PBC in many people.
• Elafibrinor (Iquirvo) or Seladelpar (Livdelzi): Your provider may add or switch to these drugs if ursodeoxycholic acid is not enough or if you cannot take it.

You will likely need to take these medicines for life.

Medicines and steps to manage symptoms

Your care team can also help you:

• Treat itching
• Manage fatigue
• Protect your bones and lower your risk of osteoporosis and fractures
• Replace fat-soluble vitamins (A, D, E and K) if needed
• Manage high cholesterol
• Make healthy lifestyle choices, including avoiding alcohol and following a liver-friendly diet

Your provider will monitor your blood tests and symptoms over time and adjust your treatment as needed.

With early diagnosis and modern treatment, many people with PBC can have a normal life expectancy, especially with early diagnosis and good response to treatment.

Possible complications

Without proper treatment and follow-up, PBC can lead to serious health problems, such as:

• Liver fibrosis and cirrhosis 
• Liver failure
• High blood pressure in the vein that connects with the liver (portal hypertension)
• Increased risk of liver cancer 
• Kidney problems
• Higher risk of infections

If your liver is badly scarred or starts to fail, you may need a liver transplant.

Because bile helps you absorb fats and fat-soluble vitamins, PBC can also cause:

• Trouble absorbing fats, which can cause greasy stools and weight loss
• Vitamin A, D, E and K deficiencies
• Thinning and weakened bones (osteoporosis) and a higher risk of fractures

When to see a specialist

Talk with your primary care provider or ask for a referral to a gastroenterologist (digestive system specialist) or hepatologist (liver specialist) if:

• Your liver blood tests stay abnormal
• You have been told you might have PBC
• You have symptoms that don’t get better
• You already have PBC and your symptoms are getting worse

It’s important to have ongoing support from a specialist to manage primary biliary cholangitis.

How Banner Health can help

At Banner Health, our gastroenterology and liver specialists care for people with PBC and other autoimmune liver diseases. Your team will work with you to:

• Confirm your diagnosis
• Create a personalized treatment plan based on your disease stage and goals
• Monitor your liver health over time and adjust your treatment if needed
• Coordinate advanced liver care, including transplant evaluation, if it becomes necessary

Take the next step

Banner Health offers expert care for primary biliary cholangitis and other autoimmune liver diseases. Our specialists focus on early diagnosis, personalized treatment and long-term liver health.

If you have symptoms of PBC, abnormal liver tests or questions about your liver health, we are here to help. Find a Banner Health liver specialist near you.