Primary biliary cholangitis (PBC) is a liver disease that damages the tiny canals in your liver called bile ducts, making it tough for bile to flow. Bile is a fluid your liver makes that helps your body digest fat, absorb vitamins, and get rid of cholesterol and toxins. A backup of bile harms liver tissues and leads to cirrhosis, where scar tissue replaces liver tissue. This affects how well your liver works.
There are four stages of PBC:
- Stage 1 (portal): This stage involves changes in the bile ducts and inflammation around the portal area of the liver.
- Stage 2 (periportal): In this stage, scar tissue forms (fibrosis), and there’s inflammation around the structures that carry blood vessels and bile ducts.
- Stage 3 (septal): Here, there’s fibrosis along the thin walls (septa) within the liver and active inflammation within these walls.
- Stage 4 (biliary cirrhosis): This is the advanced stage, where the liver has various-sized nodules and different levels of inflammation.
Primary Biliary Cholangitis Causes
Experts don’t fully understand what causes PBC, but they think it’s a mix of genetic and environmental factors.
In PBC, white blood cells called T cells gather in the liver and mistakenly attack healthy cells lining the bile ducts. Normally, these cells defend against germs, but in PBC, they harm the liver cells instead. This inflammation spreads to other liver cells, causing damage and eventually leading to cirrhosis.
Although you don’t directly inherit PBC through specific genes, the disease seems to run in families, so there may be a genetic connection. This means if one family member has PBC, others in the family may have a greater chance of getting it, too.
Primary Biliary Cholangitis Risk Factors
PBC can affect anyone, but some people are more likely to get it. Here are the things that raise your chances of getting the disease:
Sex. Women and people assigned female at birth (AFAB) are 9 to 10 times more likely to get PBC.
Age. Most people diagnosed with PBC are between 30 and 60 years old.
Family history. Having a family member with PBC boosts your odds of also getting the illness.
Background. PBC mainly affects people with a northern European background (Scotland, Scandinavia, and Northeast England).
Primary Biliary Cholangitis Symptoms
You probably won't have any symptoms in the early stages of PBC; they usually begin to appear as the disease advances. Most people with PBC feel exhausted and weak and have itchy skin. These symptoms can be mild or serious. Researchers have found that serious symptoms at an earlier stage are a sign that the disease will get worse more quickly. Later-stage PBC symptoms include:
- Skin and eye yellowing (jaundice)
- Dry eyes and mouth
- Pain in the upper right part of your stomach
- Spleen swelling (splenomegaly)
- Bone, muscle, or joint pain
- Feet and ankle swelling
- Fluid buildup in your belly (ascites)
- Fatty growths on the skin (xanthomas)
- Darkened skin (hyperpigmentation)
- Weak, brittle bones (osteoporosis)
- High cholesterol
- Diarrhea
- Greasy stools (steatorrhea)
- Underactive thyroid (hypothyroidism)
- Weight loss
- Internal stomach and esophagus bleeding due to enlarged veins (varices)
Primary Biliary Cholangitis Diagnosis
To diagnose PBC, your doctor will first examine you and ask about your medical history. Then they’ll order different tests, including:
Blood tests. A blood sample could show signs of the illness, like certain antibodies and liver enzymes that are higher than normal.
Imaging tests. These may include an abdominal ultrasound, MRI, and other specialized imaging tests to examine your liver and biliary system in detail.
Liver biopsy. Your health care team will confirm a PBC diagnosis using a thin needle to remove a small tissue sample of your liver and examine it under a microscope.
Primary Biliary Cholangitis Treatment
There’s no cure for PBC, but you can manage the condition with medication and a liver transplant if necessary. If your PBC doesn't respond well enough to ursodeoxycholic acid, your doctor may add other drugs to your treatment plan such as fibrates, steroids, and PPAR agonists. These drugs help ease inflammation and itching. Some help your liver to make less bile.
PBC medications
Your doctor will likely prescribe bile salt, ursodeoxycholic acid (UDCA), a medicine that removes bile from your liver and reduces liver damage. They might also have you take obeticholic acid (Ocaliva) with UDCA if your PBC doesn't respond to UDCA alone. Two new PPAR agonists, elafibranor (Iqirvo) and seladelpar (Livdelzi), are approved for use with UDCA as well.
Obeticholic acid (Ocaliva) also has been prescribed for people with PBC who didn't have cirrhosis of the liver. But the FDA announced in late 2024 that it had identified cases of serious liver injury in those who had been treated with the medication. Your doctor should monitor your liver tests frequently if you're being treated with Ocaliva to ensure your liver function isn't worsening.
You could also get treatment to relieve other symptoms, like antihistamines or ultraviolet light therapy for itching and vitamin supplements for low vitamin levels and osteoporosis.
Liver transplant
If your liver continues to get weaker, you could need a liver transplant. Transplants for PBC tend to have good results, and you’ll have a normal life span. Even after surgery, your symptoms could come back, but they’ll advance more slowly.
Primary Biliary Cholangitis Lifestyle Changes
Living with PBC means keeping yourself and your liver healthy for as long as possible. You can do this by making changes in your daily life, such as:
- Not smoking, drinking alcohol, and using drugs
- Cutting back on processed, packaged, high-sodium, and high-fat foods
- Eating more whole foods
- Exercising, including cardio and weightlifting
- Not eating raw shellfish (it can carry bacteria that’s dangerous for people with liver disease)
- Talking to your doctor before taking new medicines or dietary supplements
- Drinking lots of water
- Lowering stress
- Taking care of your skin
- Visiting the dentist regularly
Primary Biliary Cholangitis Complications
Damage to your liver can trigger further health problems, many of them serious. These complications include:
- Liver scarring (cirrhosis)
- Higher pressure in your portal vein (portal hypertension)
- Enlarged veins (varices)
- Enlarged spleen (splenomegaly)
- Gallstones and bile duct stones
- Liver cancer
- Weak, brittle bones (osteoporosis)
- Low levels of vitamins A, D, E and K
- Personality changes and problems with memory and concentration (hepatic encephalopathy)
- A higher chance of thyroid, skin, and joint disease
- Problems breaking down and absorbing fats (fat malabsorption)
Primary Biliary Cholangitis Outlook
Early diagnosis and treatment can help to control your symptoms. And it can ward off the later stages of the disease and further health problems. You’ll likely live a normal life but will probably feel tired a lot.
If you have an aggressive form of PBC or received a diagnosis at a later stage, you’ll need a liver transplant. With a successful transplant, the 10-year survival rate is excellent at 65%.
Questions to Ask Your Doctor
Before your next doctor’s appointment, take some time to write down a list of questions about your condition. Here are some ideas:
- How serious is my liver damage?
- What treatments do you suggest?
- Will treatment stop the disease from getting worse?
- Could my treatment cause side effects?
- Should I take any supplements?
- Should I make changes to my diet?
- Is my liver damage serious?
- Do I need a liver transplant?
- How can I learn more about my condition?
Primary Biliary Cholangitis Resources and Support
It can be tough managing a lifelong illness, especially one without a cure. Think about reaching out to family and friends for support. They can help keep your spirits up, and when you’re tired from your illness, manage day-to-day responsibilities like grocery shopping, laundry, or cooking. If you want to talk to others living with the same condition, try a support group. You can find online and in-person groups through organizations such as the American Liver Foundation.
Show Sources
Photo Credit: E+/Getty Images
SOURCES:
Cleveland Clinic: “Primary Biliary Cholangitis (PBC).”
Mayo Clinic: “Primary biliary cholangitis.”
American Liver Foundation: “Primary Biliary Cholangitis (PBC).”
Pandit, S., Samant, H. Primary Biliary Cholangitis. StatPearls Publishing, 2024.
