Primary biliary cholangitis (PBC) is a disease that destroys the bile ducts, the parts of your liver that make the digestive fluid bile. Bile assists the liver as it helps your body digest food and removes toxins from your blood. It moves into the liver through the hepatic bile ducts. When these bile ducts become damaged and no longer move bile into the liver, liver damage occurs.
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What Is Primary Biliary Cholangitis?
Your liver helps your body digest food and removes toxins from your blood. Bile, a fluid that your liver makes, helps with these actions.
Bile moves into your body through small bile ducts inside your liver known as the intrahepatic bile ducts.
Primary biliary cholangitis (PBC), formerly known as primary biliary cirrhosis, is a disease that destroys these bile ducts over time. When bile can no longer move through the ducts, liver damage occurs.
How common is primary biliary cholangitis?
PBC is not a very common disease.
It affects about:
- 65 out of 100,000 U.S. women.
- 12 out of 100,000 U.S. men.
What causes primary biliary cholangitis?
It's not clear why some people get PBC. Doctors think it's an autoimmune disease in which the body's own immune system attacks the liver.
Researchers don't think parents pass PBC to their children through gene changes, but it sometimes occurs in several family members
Primary biliary cholangitis risk factors
PBC is more common in women than men, especially women over the age of 40.
If you have PBC, you're at higher risk for other health issues like:
- Cirrhosis and complications.
- High cholesterol, a fatty substance in the blood that causes harmful plaque to form in the arteries.
- Liver failure, or end-stage liver disease.
- Osteoporosis, or thinning bones.
Complications of primary biliary cholangitis
If PBC goes untreated, it can lead to serious and potentially life-threatening complications over time.
Consequences include:
- Cirrhosis of the liver — Over time, healthy liver tissue is replaced by scar tissue, reducing liver function. This stage is known as cirrhosis, and it marks a major turning point in disease severity.
- Complications beyond the liver — Patients may develop osteoporosis, high cholesterol, and vitamin deficiencies due to poor bile flow and nutrient absorption.
- Debilitating symptoms — Untreated PBC can cause fatigue, pruritus (itching), dry eyes and mouth, and abdominal discomfort, which can significantly affect quality of life.
- Increased risk of death — Without medical management, the risk of liver-related death increases, especially in patients who progress to cirrhosis and liver failure.
- Liver damage — PBC causes the immune system to attack small bile ducts in the liver. Without treatment, it leads to cholestasis (bile buildup), which progressively damages liver tissue.
- Liver failure — As cirrhosis worsens, the liver may no longer perform essential functions like filtering toxins, producing proteins, or regulating digestion — leading to end-stage liver disease.
What Are the Signs and Symptoms of Primary Biliary Cholangitis?
If you have PBC, you might not know it. Some people have no symptoms at all.
As liver damage worsens over time, you might notice symptoms such as:
- Dark-colored urine.
- Fatigue, or feeling extremely tired.
- Itchy skin.
- Jaundice, or a yellow color in your eyes and skin.
- Lack of appetite or weight loss.
- Pain or swelling in your belly.
- Small yellow bumps on your skin.
When should I see a doctor about my primary biliary cholangitis symptoms?
You should see your primary care provider as soon as you notice any PBC symptoms, or if you have risk factors and abnormal liver tests. Early detection is key to slowing progression and preserving liver function.
How Do You Diagnose Primary Biliary Cholangitis?
Doctors might find out you have PBC during a blood test for another condition.
Your doctor will do an exam and ask if anyone else in your family has PBC.
Tests to diagnose primary biliary cholangitis
- Blood test — If they suspect you have PBC, your provider may test your blood for antimitochondrial antibodies. People with PBC have these antibodies in their blood.
- Liver biopsy — In some cases, doctors will remove a small sample of liver tissue to study it under a microscope.
How Do You Treat Primary Biliary Cholangitis?
Doctors can't cure PBC. But they can treat you to help stop — or at least slow down — further liver damage.
The goal of treating PBC is to prevent liver damage. You'll see your provider for routine physical exams and blood tests to check your liver's function.
Lifestyle changes to treat PBC
Healthy habits are especially vital when you have PBC.
Your doctor will suggest that you:
- Avoid alcohol.
- Eat high-calcium foods.
- Exercise.
- Limit salt.
Medicine to treat primary biliary cholangitis
Your doctor might prescribe certain medications to help slow liver damage from PBC.
These drugs include:
- Obeticholic acid — Which may help decrease inflammation in the liver.
- Ursodiol — An acid that helps break down cholesterol in the liver.
Some people with PBC don't have enough vitamins A, D, E, and K in their bodies because the liver can't absorb them. You might take supplements to help replace these vitamins.
Surgery to treat PBC
Over time, PBC may damage the liver so much that you experience liver failure or end-stage liver disease.
Transplant surgery, in which doctors replace a diseased liver with a donated healthy one, is the only treatment for liver failure.
Why Choose UPMC for Primary Biliary Cholangitis Care?
At UPMC, our liver experts:
- Care for all types of liver conditions with treatment options tailored to you.
- Develop advanced therapies for people with PBC.
- Work closely with the UPMC liver transplant team, a national leader in living-donor liver transplants.
Learn more about liver transplants and living-donor transplants at UPMC.
