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Finding out that you have a chronic condition, such as primary biliary cholangitis (PBC), can be stressful. Learning about the disease and possible treatments can make it easier to cope with your diagnosis.
A PBC diagnosis is often a surprise
If your diagnosis of PBC was discovered after you had routine blood tests that can indicate a problem with the liver, you’re not alone. Around 60% of people diagnosed with PBC had no symptoms of PBC before the diagnosis.
The vast majority of people with PBC are women, usually in their 50s or 60s. Although there is no cure, the prognosis is excellent when PBC is diagnosed early and responds to treatment. With treatment, PBC typically progresses slowly. People are often able to carry on with their lives for many years or decades after their diagnosis. Even when PBC is diagnosed at later stages, the treatments available help alleviate symptoms and slow disease progression.
What is PBC, exactly?
PBC is a chronic autoimmune disease that inflames and destroys small bile ducts in the liver. Without treatment, PBC may lead to cirrhosis of the liver or liver failure.
Here are definitions of some terms related to PBC to help you better understand your condition:
- Autoimmune disease. When the body’s immune system mistakenly attacks healthy tissue and cells.
- Bile. A yellowish digestive fluid made by the liver. The B in PBC is for “biliary,” which means “related to bile.”
- Bile ducts. Small tubes that bring bile from the liver or gallbladder to the small intestine.
- Cholangitis. Inflammation of the bile ducts that can injure the liver. “Cholangitis” is the C in PBC.
- Cirrhosis. Permanent scarring of liver tissues, which can lead to liver failure.
- Hepatitis. An inflammation of the liver.
- Liver failure. A life-threatening condition where the liver can no longer perform important functions such as filtering out toxic substances and making bile or other proteins your body needs.
PBC treatment is critical
If you are symptom-free, you might not think it’s necessary to start treatment. However, healthcare professionals advise beginning treatment immediately, even if you don’t have symptoms. Studies show that without treatment, the probability of remaining in the early stage of the disease is only 29% after four years.
People with PBC who do not get treatment are at higher risk of disease progression. Studies show that people with PBC who do not get treatment can have excessive fibrosis — scar tissue that builds up in the liver — after two years. After four years, 50% of people with untreated PBC had hepatitis and progressed to developing cirrhosis.
Monitoring your PBC
You’ll likely have regular medical tests and exams to monitor your liver health.
Physical exams
You will likely have regular exams to check for an enlarged liver or spleen or signs of advanced liver disease such as yellowing of the skin and eyes, called jaundice, swollen feet and ankles, or intestinal bleeding. Nail issues or scratch marks from itching on the arms and legs also are assessed.
Blood tests
Your healthcare team will likely perform these tests to gauge your levels of:
- Antimitochondrial antibodies (AMAs). This test is used to diagnose PBC. AMAs are substances that impact the cells of the liver. Finding AMAs in the blood is a very reliable sign of PBC. About 90% to 95% of people with PBC have AMAs in their blood. AMAs are rarely found in people without PBC.
- Bilirubin. This test measures the levels of bilirubin — a waste product processed by the liver — in the blood. High levels of bilirubin may indicate liver or bile duct problems.
- Alkaline phosphatase. High levels of this enzyme that’s made in the bile ducts can suggest inflammation in the bile ducts.
- Cholesterol. Many people with PBC have increases in total cholesterol levels.
When you’re first diagnosed with PBC, you’ll likely have some tests every three months. If your results are consistently within the standard range, your healthcare team may reduce testing to once or twice a year.
Imaging
Although the effects of PBC are challenging to see with an X-ray, specialized imaging can be helpful. Some imaging tests you may have include:
- Live elastography. This test measures the stiffening or scarring in the liver. The imaging expert uses an ultrasound-like scanner, such as FibroScan, on the outside of the body.
- Magnetic resonance elastography (MRE). MRE combines an MRI with sound waves to visually map internal organs. The test can detect stiffening in the liver.
- Endoscopy. During this nonsurgical procedure, an endoscope — a flexible tube with a camera attached — is inserted through the mouth to examine the digestive tract. The test may be recommended every few years for people with cirrhosis and is performed under sedation.
- Endoscopic retrograde cholangiopancreatography (ERCP). This procedure combines endoscopy and X-rays to discover and treat problems of the bile and pancreatic ducts. It is rarely needed in individuals with PBC.
Liver biopsy
A liver biopsy is a procedure where a small sample of tissue is removed from the liver with a needle. Local anesthesia is used before the procedure to numb or freeze the area. You may have a biopsy if:
- If your healthcare professional suspects you have PBC, but the blood test for AMA is negative.
- You want to know the stage of the disease. Unlike other diseases, PBC stages usually aren’t particularly important in determining treatment. But knowing the stage of the disease may help your health professional determine whether additional testing or alternate treatment is needed.
Treatments for PBC
Several treatments help people with PBC reduce symptoms and disease progression. The most common treatment is ursodeoxycholic acid (UDCA). Most people with PBC take UDCA daily, usually in pill form. UDCA is a naturally occurring, nontoxic bile acid that dissolves with water and has few side effects. The treatment is the same for all stages of the disease.
If you are intolerant to UDCA, or UCDA doesn’t work for you after 6 to 12 months, your healthcare team might recommend the following:
- Obeticholic acid (Ocaliva). This semisynthetic bile acid equivalent also is used to help people with advanced liver conditions like cirrhosis.
- Elafibranor (Iqirvo). This lipid regulator and anti-inflammation agent has recently been approved by the Food and Drug Administration for treatment of PBC.
- Seladelpar. This lipid regulator and anti-inflammation agent is scheduled to undergo review with the Food and Drug Administration in August of 2024. It may be approved for use at that time.
- Liver transplant. In the late stages of PBC, where the liver has developed significant cirrhosis, a liver transplant may be an option.
Clinical trials also may be available for people with PBC. Ask your healthcare team for more information.
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