What is primary biliary cholangitis (PBC)?
Primary biliary cholangitis (PBC) is a lifelong autoimmune liver disease. In PBC, the body’s immune system mistakenly attacks the small bile ducts inside the liver. Over time, this damage can make it harder for bile to flow normally. This can lead to scarring of the liver, called fibrosis, and in some people may eventually cause cirrhosis, liver failure, or the need for a liver transplant.
PBC is not caused by alcohol use, and it is not contagious. Most people with PBC will live for many years with proper treatment and monitoring.
What are the symptoms of PBC?
Some people have no symptoms from their PBC. Others may develop symptoms that affect daily life.
Common symptoms include:
- Itching (pruritus).
- Fatigue (feeling very tired).
- Trouble sleeping because of itching.
- Dry eyes or dry mouth.
- Discomfort in the upper right side of the abdomen.
Many people living with PBC report that itching and fatigue have the greatest impact on their quality of life, even when liver tests are stable.
How is PBC diagnosed?
Your healthcare provider will use a combination of tests for diagnosis, such as:
- Blood tests.
- Liver enzyme tests.
- Antibody testing.
- Imaging studies.
- Occasionally, a liver biopsy.
One important blood test looks for antimitochondrial antibodies (AMA). Most people with PBC have a positive AMA test. However, a small number of people have PBC even though their AMA test is negative.
If your healthcare provider suspects PBC but your AMA test is negative, additional antibody tests called anti-sp100 and anti-gp210 may help confirm the diagnosis and sometimes avoid the need for a liver biopsy. If these tests are negative but PBC is still strongly suspected, a liver biopsy may still be needed.
What is the first treatment for PBC?
A new guideline from the American Gastroenterological Association (AGA) and the American Association for the Study of Liver Diseases (AASLD) strongly recommends a medication called ursodeoxycholic acid (UDCA) as the first treatment for everyone with PBC. It should be started as soon as the diagnosis is made.
Research has shown that UDCA can:
- Improve liver blood tests.
- Slow liver damage.
- Delay disease progression.
- Improve transplant-free survival.
UDCA is generally well tolerated and safe. Some people may experience mild side effects such as diarrhea, nausea, or weight gain. It is very rare for people to stop the medication due to side effects.
Important to know
Even if your liver tests do not return completely to normal, your healthcare provider may still recommend continuing UDCA because it can continue to provide important benefits.
How do healthcare providers know if treatment is working?
After starting UDCA, your healthcare provider will regularly monitor your liver tests. Many experts assess response within 6 to 12 months after treatment begins.
Your healthcare team may look at:
- Alkaline phosphatase (ALP).
- Bilirubin levels.
- Symptoms.
Other liver test results. These results help determine whether your PBC is responding well to treatment or whether additional treatments may be needed.
What happens if UDCA isn't enough?
Some people do not respond fully to UDCA or cannot tolerate it. In that situation, your healthcare provider may discuss adding or switching to newer medications called PPAR agonists, including:
- Seladelpar.
- Elafibranor.
These medications can help improve liver test results and may also reduce itching in some people. Decisions about these treatments should be made together with your healthcare team, taking into account your symptoms, liver health, goals, and preferences.
Why might I need liver stiffness testing?
Your healthcare provider may recommend a test called transient elastography (sometimes known by the brand name FibroScan®).
This test measures liver stiffness, which can help estimate how much scarring is present in the liver. It is painless and does not require needles.
The results may help your healthcare provider:
- Better understand your risk of future liver problems.
- Decide how closely you should be monitored.
- Determine whether treatment changes should be considered.
What if itching becomes severe?
Itching can be one of the most challenging symptoms of PBC. It can interfere with sleep, work, and daily activities.
The guideline suggests that linerixibat may be an option for people with moderate-to-severe PBC-related itching.
In clinical studies, linerixibat helped some people experience less itching, although benefits varied from person to person.
Possible side effects of linerixibat include:
- Diarrhea.
- Abdominal pain.
- Increased bowel movements.
Because people value symptom relief differently, the decision to use linerixibat should be made after talking with your healthcare team.
Can PBC come back after a liver transplant?
Although liver transplantation can successfully treat advanced PBC, the disease can sometimes return after transplant.
The AGA-AASLD guideline suggests continuing treatment with UDCA after transplant to help reduce the risk of PBC coming back and impacting the health of the transplanted liver.
Living well with PBC
Most people with PBC lead active, fulfilling lives. Successful management often includes:
- Taking medications as prescribed.
- Keeping follow-up appointments.
- Monitoring liver tests regularly.
- Reporting new or worsening symptoms.
- Discussing itching, fatigue, and quality-of-life concerns with your care team.
PBC treatment is becoming more personalized. Healthcare providers now look at more than blood tests. They consider your symptoms, liver health, age, and personal goals when making treatment decisions.
Questions to ask your healthcare team
- How advanced is my PBC?
- Should I start UDCA?
- How will we know if my treatment is working?
- Do I need liver stiffness testing?
- What should I do if I have severe itching or fatigue?
- Would I benefit from additional treatments beyond UDCA?
- How often should I have follow-up visits and blood tests?
Empowering you with information from the latest AGA/AASLD Clinical Practice Guideline on Management of Primary Biliary Cholangitis (Levy C et al., 2026)
Reviewed by
Elizabeth J. Carey, MD
Gastroenterologist and transplant hepatologist, Mayo Clinic, Phoenix, AZ
Aparna Goel, MD
Clinical Associate Professor, Medicine - Gastroenterology & Hepatology, Stanford Medicine, Redwood City, CA
September 2026