What Is Primary Biliary Cholangitis?

Medically Reviewed by Minesh Khatri, MD on July 20, 2023
8 min read

Primary biliary cholangitis (PBC) is a rare liver disease. It blocks and destroys bile ducts in the liver.  Doctors used to call it “primary biliary cirrhosis.”

“Biliary” means bile. That’s a digestive fluid that helps your body get rid of extra fats, like too much cholesterol, and other things your body doesn’t need.

“Cholangitis” is the inflammation of the liver's bile ducts. 

If you have this condition, bile may not be able to move out of the liver, so the things that bile gets rid of can build up or move into other parts of the body. That can scar and damage organs. It’s progressive, which means it can get worse over time. 

There’s another rare liver condition called primary sclerosing cholangitis (PSC). While it has a similar name to PBC, the conditions have several differences. PBC mostly affects middle-aged people, while PSC can impact all ages. Also, 90% of people with PBC are women, but it’s mainly men who have PSC. Finally, PBC affects bile ducts inside the liver, while PSC is a disease of bile ducts both inside and outside of the liver. 

Only about 400 out of 1 million people get this condition. Most often, it affects women between ages 30 and 60.

Sometimes it runs in families. You could be more likely to get it if your mom, dad, brother, or sister – especially an identical twin – has the disorder.

Doctors don’t know what causes primary biliary cholangitis. Researchers are studying how genes, changes to the immune system, and other things may play a role.

People with this condition have very low levels of certain white blood cells called circulating T cells. These cells help your body fight infections. They may work differently in people with primary biliary cholangitis. It’s not yet clear how or if these differences trigger the liver disorder or its symptoms.

Another theory is that the disease is an immune system problem.

You might not have symptoms at first. In more than 60% of people who have this condition, doctors find it unexpectedly when someone gets blood tests to check how well their liver works.

If you do have symptoms, the first and most common ones are:

  • Itching due to a buildup of bile in the body
  • Feeling tired all the time (fatigue)

Later, you may have:

  • Darkened skin
  • Dry eyes and mouth
  • Joint, muscle, or bone pain
  • Upper right-side belly pain
  • Weight loss
  • Swelling in your feet and ankles (edema)
  • Diarrhea
  • Fat buildup on the skin around the eyes, eyelids, or in the creases of the palms, soles, elbows, or knees

You may also have yellow skin and whites of the eyes (jaundice) due to a buildup of bile in the body.

Over time, your liver tissue can scar and harden. Other complications can start as the liver stops working because of scarring. This is called cirrhosis. Gallstones and bile duct stones can form, causing pain and infections.

Other complications of PBC include:

  • High blood pressure in the liver vein (portal hypertension). This can lead to fluid buildup in your feet, ankles, legs, and belly. Your spleen may swell.
  • Hepatic encephalopathy. Toxins that the liver usually removes start to build up in that organ and eventually the brain. This can cause confusion, personality changes, and even a coma.
  • Swollen blood vessel (varices) in the esophagus and stomach. These can burst and cause serious, life-threatening bleeding.
  • Vitamin and metabolic deficiencies. When the bile ducts become blocked, the liver can no longer filter substances, such as cholesterol and other fats, as it normally would. This changes the way the body breaks down fats and the vitamins A, D, E, and K. As a result, your body can’t use nutrients the way it once did. Osteoporosis (brittle, weak bones) can happen when your body has problems using vitamin D and calcium.
  • Other autoimmune disorders. It's not unusual to find other autoimmune diseases diagnosed in patients with PBC. Those may include such conditions as Sjogren’s syndrome (dry eyes and mouth) and autoimmune thyroid disease. 
  • Steatorrhea. When bile can’t get to the small intestine, your body can’t absorb fat properly and you get this condition. Fat then builds up in your stools, creating loose, greasy, and foul-smelling bowel movements.
  • High cholesterol. As many as 80% of people with PBC also have high cholesterol.

Your doctor will give you a physical exam and ask questions about your family and medical history. You may also get these blood tests:

  • Alkaline phosphatase, a test for increased liver enzymes
  • AMA, a test to see if your body is making antibodies that attack your mitochondria –your cells’ energy powerhouses. Your doctor may call these “antimitochondrial antibodies.”

You will also need imaging tests, such as an ultrasound, MRI, or X-ray of your bile ducts.

Your doctor may also want to do a liver biopsy, where they take a small sample of tissue to test in a lab.

You have primary biliary cholangitis if you have at least two of the following:

  • High levels of alkaline phosphatase
  • Antimitochondrial antibodies
  • Signs of the disease on a liver biopsy

If your doctor has diagnosed you with PBC, you’ll probably have lots of questions about the condition. Here are several questions to have ready for your next appointment:

  • In which stage of the disease am I?
  • How will PBC impact my physical health?
  • How will it affect my day-to-day life (work, family, etc.)?
  • Should I make any changes to my lifestyle?
  • Will I need more tests?
  • Which treatment do you suggest? What are the possible side effects?
  • Will I need a liver transplant?
  • Are there alternative therapies you’d suggest?
  • What other health conditions could I form along with PBC?
  • Can I stop the disease from getting worse?
  • Can you recommend any clinical trials on PBC?
  • Can you suggest a PBC support group?

PBC doesn’t have a cure, but you can take medicine to slow the rate at which it gets worse and prevent other health problems. The main treatment for PBC is a drug called ursodiol. It’s a natural form of bile that can help your liver work better and help you live longer. You take it as a pill, unless you get a new liver in a transplant. There are few reported side effects from the drug.

If you can’t take ursodiol, or it doesn’t work well enough for you, your doctor may prescribe obeticholic acid (Ocaliva) along with, or instead of, ursodiol.

You could also take fibrates (Tricor) with ursodiol to ease liver inflammation and itching. Budesonide is a corticosteroid that you may also take with ursodiol to treat the inflammation of PBC without the full-body side effects that are common with other drugs of this type.   

Your doctor may prescribe other treatments to ease symptoms. They may include:

  • Medications to relieve itching, such as cholestyramine or colestipol
  • Drugs to lower blood pressure in the veins
  • Diuretics, or water pills, to remove extra fluid from your body
  • Plasmapheresis, a procedure to remove unwanted substances from the blood
  • Vitamin supplements to treat vitamin K, A, D, calcium, and iron deficiencies
  • A low-fat diet with medium-chain triglycerides to boost calorie intake
  • Artificial tears for dry eyes

If you have liver failure or complications that can no longer be effectively treated, your doctor may recommend that you get on the list for a liver transplant. This is when doctors replace your unhealthy liver with one from a donor.

Taking care of yourself is essential to your quality of life with PBC. Here are some ideas:

Food. Eat a healthy, well-balanced diet that’s high in fruits in vegetables and low in sugar and fat. Eating small meals more often instead of three large ones will help you meet your calorie needs without overburdening your liver.

When you have PBC, it’s critical to steer clear of anything that could cause an infection. Stay away from raw or undercooked meats or fish, including oysters and other raw shellfish, and unpasteurized dairy products since they can carry bacteria that’s unsafe for people with PBC. Be sure to wash all produce thoroughly.

Also, too much salt in your diet can cause swelling and a buildup of fluid in spaces in your abdomen (ascites) This means you’ll need to choose low- or reduced-sodium foods.

Exercise. Make it a point to exercise most days of the week. Walking and using light weights can help lower your chances of bone loss (osteoporosis) and boost bone density.

PBC usually advances slowly: It takes around 15 to 20 years to reach the final stage. It’s possible to delay or even ward off the later stages of the disease with early diagnosis and treatment. Medicine helps to control many symptoms, and the disease may not affect your day-to-day life for years. On average, people with PBC live for an average of 10 years once they start to have symptoms. 

A later diagnosis or more aggressive form of the disease means you’ll need a liver transplant. The outlook for people with PBC who have this surgery is very good, although it’s possible for the disease to return. The 10-year survival rate for people who’ve had a successful liver transplant is 65%.

Fatigue is a common symptom of PBC, which can make you feel tired all the time. Ask friends or family for (or accept) help if you need it. It could come in the form of grocery shopping, laundry, cooking, or other daily tasks. Don’t be afraid to tell others what you need.

Support groups are also available that let you share your experiences with others on a similar path. Look for groups for people with liver disease, either online or in person.

If you have this condition, you should avoid drinking alcohol and never use illegal drugs. These things can further damage your liver.

Tell your doctor before you use any type of over-the-counter medications or remedies, especially herbs and supplements. Many substances can affect how your liver works and may interfere with your treatment and outcome.

Liver cancer is a concern in people who develop scarring of the liver, called cirrhosis. Talk to your doctor about what tests you should get. They may want to check you for the disease every 6 to 12 months.

The first step in taking care of your mental and emotional health is to talk to your primary care doctor. They may refer you to a mental health professional.

Also, focus on things that bring you joy, like watching your favorite TV show or going for a walk. If you still feel anxious, take 15 minutes out of each day for “worry time.” Just be sure it’s not close to the time you go to bed. Use this time to think about all your worries and even write them down. Then set them aside and come back to the present moment.

You can find more information on primary biliary cholangitis from:

  • American Liver Foundation
  • National Institute of Diabetes and Digestive and Kidney Diseases
  • National Organization for Rare Disorders