What Is Primary Biliary Cholangitis?

Written by Alyson Powell Key, Michelle Bourg
Medically Reviewed by Melinda Ratini, MS, DO on August 04, 2025
15 min read

Primary biliary cholangitis (PBC) is a rare liver disease. “Biliary” means bile, which is a digestive fluid that helps your body with digestion and absorbing nutrients. It also helps get rid of extra fats, like too much cholesterol, and other things your body doesn’t need. If you have PBC, your body begins to damage bile ducts in the liver, which causes inflammation. “Cholangitis” is the inflammation of the liver's bile ducts. Doctors used to call it “primary biliary cirrhosis” because it can cause scarring of the liver, especially if it is not treated early.

While PBC is a progressive disease that gets worse over time, it is often caught before symptoms even start and there are effective treatments for managing symptoms and slowing the disease’s progression.

Who gets it?

Only about 3 in 100,000 people get this condition. Most often, it affects women between ages 30 and 60. Sometimes it runs in families. You may  be more likely to get it if your mom, dad, brother, or sister – especially an identical twin – has the disorder.

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. 

PBC is an autoimmune disease, which means it is caused by the immune system. The immune system’s job is to defend the body from bacteria, viruses, and other harmful things. In autoimmune diseases, the immune system is overactive and begins to attack healthy tissue in our bodies. PBC is the most heritable (meaning it is passed through genes) autoimmune disease. It is more common in families where a parent or sibling has the condition and in identical twins (who share 100% of their DNA).

The current theory among researchers is that environmental triggers cause the immune system to begin attacking itself in people who are genetically prone to the condition.

Environmental triggers are something you are exposed to in everyday life that can cause your body to develop a disease, particularly if you have a genetic predisposition to the condition. In the case of PBC, triggers that have been identified so far include toxic waste, cigarette smoking, nail polish, hair dye, and some kinds of bacteria. 

There are two main types of PBC: 

AMA-positive PBC. More than 90% of people with PBC have this variant or type, which is also called “classic.” The main antibodies involved are called antimitochondrial antibodies (AMA), which attack the powerhouse of the cell, the mitochondria.

AMA-negative PBC. If someone with PBC doesn’t have AMA in their blood, they will have circulating antinuclear antibodies (ANA), which attack the nuclei of cells.

The symptoms of the disease are pretty much the same no matter what type you have, although there is some evidence suggesting that the involvement of antinuclear antibodies may play a role in how the disease progresses, but more research is needed. For now, the presence of these different antibodies is mostly used to diagnose PBC (see “Diagnosis” below). 

You might not have symptoms at first. More than 60% of people who have PBC  find out when their  doctor runs routine blood tests or is checking to see how your liver is functioning. Symptoms usually begin five to 20 years after diagnosis. 

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:

  • Jaundice (yellowing of the skin and whites of the eyes)
  • Joint, muscle, or bone pain
  • Upper right-side belly pain
  • Darkened skin
  • Dry eyes and mouth
  • 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

When bile ducts are damaged, people have higher levels of an enzyme called alkaline phosphatase. If routine blood tests find a high level of alkaline phosphatase in your blood, PBC could be one of the causes.  Your provider will order more specific bloodwork to look for the presence of AMA, or antimitochondrial antibodies, which are most commonly found in someone with PBC. If you have AMA circulating in your body, you will usually not need a liver biopsy to confirm the diagnosis.

They may also test for ANA, or antinuclear antibodies, but without the presence of AMA, your doctor will likely order a liver biopsy. 

A liver biopsy is a simple, short procedure that does not require anesthesia. Your provider will insert a needle through your skin to your liver and remove a small piece of  it to look at under a microscope. In the lab, the technicians will be looking for signs that your bile ducts are inflamed (swollen) or damaged, which means it is likely that you have PBC. 

Your doctor will also ask about your family medical history, perform a physical exam, and may order imaging tests, such as an ultrasound, MRI, or X-ray of your bile ducts to rule out other possible causes.

You will be diagnosed with 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 some 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?

When you are living with PBC, it is important to have a care team you trust that will include a liver specialist. This could be a hepatologist (a specialist of the liver and biliary system) or a gastroenterologist (a specialist in diseases of the digestive tract and liver). 

They will likely connect you to other providers such as dietitians,  who can help you learn how to nourish yourself with a balanced diet – focused on key nutrients and vitamins – that will support your liver and decrease the chance of complications. Your primary PBC doctor may also suggest other experts such as  autoimmune specialists, known as rheumatologists, or transplant doctors if needed. 

Resources for finding expert care

The American Liver Foundation has a comprehensive list of types of providers and how they might play a role in your care as well as a State Resources page with links to state-specific resources, including transplant and other medical centers where you can find liver specialists. 

There isn’t  a cure for PBC yet, but you can take medications that will help slow the rate at which it progresses and prevent and treat other health problems related to it. 

Medications

Ursodeoxycholic acid (UDCA). The main treatment for PBC is UDCA, also 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. There are few reported side effects from the drug.

Elafibranor (Iqirvo). The newest FDA-approved treatment for PBC is elafibranor, which can help if UDCA is not working well enough. Researchers don’t know exactly how it treats PBC, but it acts on a part of your body involved in how you process bile. Called a PPAR agonist, elafibranor eases inflammation in your liver and helps your liver make less bile and flush out any built-up bile acid. 

Obeticholic acid (Ocaliva). If you can’t take ursodiol or it doesn’t work well enough for you, your doctor may prescribe obeticholic acid along with, or instead of, ursodiol. Obeticholic acid is a synthetic (human-made) form of a bile that your body naturally produces. 

Other treatments you may take: 

Budesonide. This 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.   

Fenofibrate (Tricor). This type of medication, known as a fibrate, acts similar to elafibrinor. Your doctor may prescribe it on an off-label basis to help ease liver inflammation and itching. A medication is “off label” when it has been approved by the FDA for other conditions but not the one you have. Doctors prescribe drugs off-label, and the FDA agrees that they can, when there is good clinical evidence that it helps your condition. You take fenofibrate in addition to UDCA. Researchers are still studying how well it works to slow PBC progression and liver-related complications. 

Seladelpar (Livdelzi). This PPAR agonist helps lower blood levels of the enzyme alkaline phosphatase (ALP).  High levels of ALP are often a sign of liver problems, although you can have normal ALP levels and still have PBC. This drug also helps your liver make less bile. If UDCA hasn't helped your PBC symptoms, it may help ease itching.  

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

Surgery

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 so that you can replace your struggling liver with one from a donor.

Most people with PBC are diagnosed before they have symptoms, which means they can begin treatment right away and significantly slow damage to their liver. That, in turn, reduces their risk for complications. Only 15% of PBC patients develop non-cancerous complications 15 years after diagnosis. When complications do develop, it is because PBC can cause your liver tissue to scar and harden, which makes it harder for your liver to do its job. This is called cirrhosis. Gallstones and bile duct stones can also form, causing pain and infections.

Other complications of PBC include:

High cholesterol. As many as 80% of people with PBC also have high cholesterol.

High blood pressure in the liver vein (portal hypertension). This can cause fluid buildup in your feet, ankles, legs, and belly. Your spleen may swell.

Swollen blood vessels (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 does. 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 conditions such 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, so it builds up in your stools, creating loose, greasy, and foul-smelling bowel movements.

Hepatic encephalopathy. When your liver isn’t working well, the toxins that it usually removes start to build up in your liver and your blood. If they reach the brain, they can cause confusion, personality changes, and even a coma. This complication only happens in late stages of the disease. 

Hepatocellular carcinoma. This is the most common type of liver cancer and can happen when the liver is damaged, particularly if someone develops cirrhosis from hepatitis B or C or PBC. Older men with portal vein hypertension or cirrhosis are the most at risk for developing it. For these patients, it’s recommended to get an ultrasound of your liver every six months.   

Taking care of yourself will make a big difference in your quality of life with PBC. Focus on:

  • Eating a well-balanced diet that’s high in fruits and vegetables and low in sugar and fat. Having small meals more often instead of three large ones will help you meet your calorie needs without overburdening your liver. Choose low- or reduced-sodium foods because too much salt in your diet can cause swelling and a buildup of fluid in spaces in your abdomen. The American Liver Foundation offers a 30-day Liver Healthy Meal Plan that can give you some ideas. 
  • Avoiding exposing yourself to bacteria because infections can make PBC worse or cause complications. Steer clear of 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.
  • Moving your body most days of the week. This is really helpful when you have PBC. It can help with any mood symptoms, such as depression and anxiety, and walking and using light weights can help lower your chances of bone loss (osteoporosis) and boost bone density.

PBC usually advances slowly: It takes about 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 will likely mean 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%.

How to protect your liver

When you have a condition that makes it harder for your liver to do its job, it's important to do everything you can to keep your liver from working too hard. One key way you can do that is to not put extra things into your body that need to be processed in the liver. This means:

  • Avoiding alcohol and never using illegal drugs. 
  • Talking to your doctor before you use any type of over-the-counter medications or remedies, especially NSAIDs (nonsteroidal anti-inflammatory drugs), herbs, and supplements. Many substances can affect how your liver works and may interfere with your treatment and outcome.

How to relieve the itch 

Pervasive and persistent itching can really impact your day-to-day quality of life, which is why it’s important to find what works for you to relieve it. 

  • Ask your doctor which treatments might help improve your symptoms. 
  • Avoid harsh or drying soaps and shower products, bathe in lukewarm water, and try Epsom salt or oatmeal baths. 
  • Moisturize well. Keep lotion in the refrigerator to add to the relief it brings when you put it on. 
  • Apply cold. Cold compresses, cool water, and cold items such as bags of peas can all bring relief. 
  • Wear comfortable clothing. 

Living with fatigue 

Fatigue is a common symptom of PBC. There is no treatment for it, but these are things you can do to live better with it. 

  • Move your body enough to keep your energy up but not so much that you burn yourself out.   
  • Protect your sleep by practicing good sleep hygiene. 
  • Don’t fight fatigue. Let yourself rest when it hits and take it into account when you create your daily schedule. Many people find fatigue is worse in the afternoon. 
  • Rely on family and friends to take care of errands and chores when you aren’t up to it.

Living with a rare and chronic disease can feel overwhelming and isolating at times, so it’s important to connect to good support, do things to bring joy into your life, and seek professional mental health support if you need it.

Here are ways to do that:

Do things you enjoy. Think about what kinds of activities make you laugh, relieve stress, or connect you to the present moment, and then incorporate them into your daily or weekly routine.

Designate 15 minutes of “worry time” each day if you are experiencing anxiety. Think through your worries and write them down. The simple act of getting them out of your head can help ease them. Looking at them will also help you identify things you can do to address them, such as asking for more support, sending your doctor questions through the portal, or engaging in stress-relieving activities that have worked for you in the past. 

Seek out professional support if you need it. Depression and anxiety are both common, and understandable, complications of living with a chronic condition like PBC. A mental health professional can be an important member of your care team and make coping with PBC much easier. Reach out to one sooner rather than later if you are struggling.

Caregivers play an important role in the management of PBC. Here are ways you can help your friend or loved one and take care of yourself. 

Learn about PBC – what it is, how it’s treated, and the ways it affects people who are living with it.

Ask how you can help. In the early days of diagnosis, it’s likely they may not know what they need, but it’s always reassuring to know that someone is ready to support you. 

Offer to accompany them to appointments. There is a lot to learn, take in, and process when you get a diagnosis of PBC. It also brings up a lot of emotions that can make it difficult to focus on and remember all of the information a provider is giving you. Having another person to take notes, remind them of questions they wanted to ask, and just be there as a support is a huge help. 

Pitch in. If your person is experiencing fatigue associated with PBC, you can help by offering to run errands or take care of tiring chores around the house. It’s also important to know that fatigue is a very real symptom of this condition. Validate their experience of it and encourage them to scale back and rest when they need to. 

Have fun with them. It’s important for anyone living with a chronic condition to continue to do things they enjoy. Take breaks from talking about PBC to do activities and outings together. 

Take care of yourself. It is hard when someone you love is sick. You might worry about them. You might have a lot on your own plate, and supporting them adds to your stress level. Make sure you are pacing yourself and getting support. It won’t be good for you or your loved one if you burn out. Often, PBC patient groups have support groups for caregivers too. 

Primary biliary cholangitis is an autoimmune condition that causes damage to your liver. It mostly affects women and is usually caught before you develop any symptoms. Early treatment of PBC can lessen or even ward off complications and serious damage to your liver. There is effective treatment for PBC and clinical trials are underway to find more. By participating in a clinical trial, you may be able to get access to cutting-edge treatment. There are a lot of things you can do to take care of yourself and live a good life with PBC. 

How rare is PBC?

PBC is very rare. Only 3 out of 100,000 people develop PBC. Most of them are women. 

What are some PBC symptoms?

It is common for people who have PBC to be diagnosed before they develop symptoms because routine blood tests can show their liver is struggling and it may be related to PBC. When people do develop symptoms, the most common ones are itching and fatigue.

Is PBC hereditary?

Yes, PBC is the most heritable (meaning it is passed down in families through genes) autoimmune disease. 

What are the tests for PBC?

Blood tests to diagnose PBC look for three things: high levels of an enzyme called alkaline phosphatase, the presence of antimitochondrial antibodies (AMA), and the presence of antinuclear antibodies (ANA). A liver biopsy is often needed to look for damaged cells in the liver. 

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