Primary biliary cholangitis, or PBC (formerly called primary biliary cirrhosis), is a chronic liver disease. That means it lasts a long time and slowly gets worse over the years. Right now, there’s no cure for PBC. Treatment focuses on slowing its progress and protecting the liver from lasting damage.

Treatment Options for PBC

Doctors think PBC is an autoimmune disease. This means the body’s immune system, which normally fights germs, mistakenly attacks healthy cells. In PBC, your immune system attacks the small bile ducts in your liver and creates inflammation. Over time, inflammation kills off healthy cells. Your liver tries to heal itself through fibrosis (making scar tissue). This tissue narrows the bile ducts and causes bile to build up.

Bile is a fluid that flows through the bile ducts into the small intestine. It’s responsible for breaking down fats and helping your body absorb vitamins and process cholesterol. Damaged bile ducts can’t carry bile to your digestive system, causing buildup in your liver and liver damage. Eventually, the scar tissue interferes with your liver function. It can also lead to permanent damage, known as cirrhosis.

Medication can help bile flow better. It can also reduce swelling in the liver and help with PBC symptoms.

Ursodeoxycholic Acid

Ursodeoxycholic acid (UDCA) is the first treatment doctors will prescribe for PBC. It’s also known as ursodiol (sold under several brand names). UDCA helps move bile through the liver into the small intestine.

According to the American Liver Foundation, UDCA is effective in more than half of people with PBC. It’s important to start taking UDCA early in PBC because it can help slow down how quickly the disease gets worse. UDCA likely won’t help with common symptoms like feeling tired and itchiness. However, it can help reduce liver scarring and may prevent the need for (or delay) a liver transplant.

According to the American Liver Foundation, UDCA is effective in over half of people with PBC.

About 5 percent of people with PBC can’t tolerate UDCA. Weight gain, hair loss, and diarrhea are possible side effects. Doctors can tell if UDCA is working by measuring your alkaline phosphatase (ALP) and bilirubin levels. The treatment isn’t working if these blood test levels don’t return to normal. At that point, it’s time to try something else.

For people whose symptoms don’t improve with UDCA, another medicine is added. Options for a second medication include:

• Obeticholic acid
• Elafibranor
• Seladelpar
• Fibrates
• Liver transplant
• Investigational treatment

Obeticholic Acid

Another treatment for PBC is obeticholic acid (Ocaliva). The U.S. Food and Drug Administration (FDA) approved obeticholic acid in 2016. It can be used with UDCA or by itself if someone can’t take UDCA. Like UDCA, obeticholic acid slows liver fibrosis or scarring while improving liver function. If you’re already itchy from PBC, obeticholic acid may make the itchiness worse.

The FDA also issued a black box warning, which is its most serious safety warning, stating that obeticholic acid may cause liver damage in people with cirrhosis. Because of this, obeticholic acid should not be used in people with advanced liver disease or liver failure. There was also a recent FDA safety update in December 2024 recommending closely monitoring liver tests, as cases of liver injury have been reported in people without cirrhosis. Your healthcare provider can help you decide if this medication is safe for you.

Elafibranor

The FDA approved elafibranor (Iqirvo) in 2024 to treat PBC. This medication works by targeting pathways that cause liver inflammation. It belongs to a group of drugs called peroxisome proliferator-activated receptor (PPAR) agonists. In studies, elafibranor helped lower ALP levels (a marker of liver damage) and helped keep the liver working well.

Elafibranor is taken once a day by mouth. Healthcare providers may suggest elafibranor be taken with UDCA in people who aren’t getting results from UDCA only. They may also prescribe elafibranor alone for people who can’t tolerate UDCA.

Common side effects of elafibranor include mild to moderate gastrointestinal issues. Some people have weight changes, abdominal pain, nausea, and diarrhea. Always tell your healthcare provider if you experience any side effects of medication.

Seladelpar

Another PPAR agonist approved to treat PBC in 2024 is seladelpar (Livdelzi). This PBC medication may be combined with UDCA or prescribed by itself.

Clinical trials have found several benefits of seladelpar for PBC management, including:

• Less reported fatigue, sleep problems, and itching
• Lower alkaline phosphatase levels
• Reduced bilirubin levels

You can take seladelpar with or without food. However, it needs to be taken four hours before or after bile acid sequestrants, which are medicines that help lower cholesterol and remove bile acids from the body. Some of the more common side effects include bloating, feeling full, headache, and nausea.

Fibrates

Fibrates are a type of medicine usually used to treat high cholesterol. They also work as PPAR-alpha agonists, which means they help turn on proteins that control fats and lower inflammation in the body.

If UDCA doesn’t work well for you, your doctor may suggest trying fenofibrate (sold under several brand names).

Researchers aren’t quite sure why fenofibrate helps treat PBC. Some studies suggest that fibrates help reduce bile acid production and target liver inflammation. Taking fibrates with UDCA may also help skin itching in PBC.

Common side effects of fibrates include sneezing, a runny nose, and abnormal lab test results. Call your healthcare provider right away if you experience sharp stomach pain, muscle pain, jaundice (yellowing of the eyes and skin), fever, chest pain, or other severe symptoms.

Liver Transplant

Because PBC is a progressive disease — meaning it continues to get worse over time — medications may eventually stop working. The result is cirrhosis and liver failure. The best way to treat liver cirrhosis and failure is with a liver transplant. This surgery replaces the diseased liver with a healthy liver from a donor.

The best way to treat liver failure is with a liver transplant. This surgery replaces the diseased liver with healthy tissue from a donor.

Most people who receive a liver transplant have a good prognosis (outlook) with PBC. However, there’s a chance that PBC might return after a transplant. Work closely with your doctors and stick to your treatment plan to avoid this complication.

Investigational Treatments

Researchers are studying other treatments for their potential to help people with PBC. One medication being studied is budesonide. Budesonide is a corticosteroid (steroid) that may be used to treat PBC in people without liver cirrhosis who did not benefit from other treatments. This medication can come with many side effects like:

• Swelling
• High blood pressure
• Mood swings
• Weight gain
• Eye problems
• Osteoporosis (weaker bones)

If your healthcare provider thinks you’ll benefit from corticosteroids, they’ll monitor for side effects and make changes as needed. According to Mayo Clinic, more research needs to be done before budesonide is recommended for treating PBC.

Treating the Symptoms of PBC

Liver damage from PBC can affect many other parts of the body. Many people experience itchy skin or symptoms of other related autoimmune diseases. Along with PBC treatments, doctors may also prescribe medications to help manage associated symptoms, like itchy skin or dry eyes.

Treatments for Itchy Skin

Intense, itchy skin is a common and uncomfortable symptom of PBC. Over-the-counter antihistamines or allergy medications block histamines — the chemicals responsible for causing itching during an allergic reaction. Diphenhydramine (Benadryl) or loratadine (Claritin) may help, especially if itchy skin keeps you awake at night. Some of these medications may cause drowsiness. You can find these medications at your local drugstore or grocery store.

Your doctor may also prescribe the following medications for itchiness:

• Cholestyramine — A medication typically used to treat high cholesterol
• Opioid antagonists (naltrexone, naloxone) — Medications that also act on the brain to stop itching sensations
• Rifampin (Rifadin, Rimactane) — An antibiotic that may block itching sensations
• Sertraline hydrochloride (Zoloft) — A selective serotonin reuptake inhibitor (SSRI) used to treat itching

These medications come with a wide range of possible side effects. Ask your doctor or pharmacist what to expect. Tell your healthcare provider if you’re experiencing side effects after starting treatment.

Treatments for Dry Mouth and Dry Eyes

PBC can also cause dry eyes and dry mouth. Your doctor may suggest using saliva substitutes, chewing gum, sucking on hard candy, and using artificial tears and eye drops like pilocarpine to help. These treatments aren’t likely to cause bad side effects, but keeping up with regular eye and dental exams is a good idea.

Treating Complications of PBC

PBC can lead to other health complications like vitamin deficiencies, bone problems, high cholesterol, and cirrhosis. Your doctor can help you manage these complications with medications and lifestyle changes.

Without enough bile in the digestive system, your body can’t properly absorb fat-soluble vitamins (vitamins A, D, E, and K). Doctors may prescribe vitamin supplements to help boost vitamin levels, like vitamin D and calcium for strong bones and vitamin K for blood clotting. Your physician may also recommend checking for low bone mass with a bone scan.

High cholesterol and xanthomas (deposits of cholesterol in the skin) can also develop in people with PBC. This is treated with the use of fibrates and other lipid-lowering agents.

When a lot of scar tissue builds up in the liver as PBC gets worse, it can lead to cirrhosis. Complications of cirrhosis include:

• Variceal bleeding — Bleeding from large veins in the esophagus
• Ascites — Fluid buildup in your abdomen
• Encephalopathy — Confusion or trouble thinking clearly, caused by toxins building up in the body
• Hepatocellular carcinoma — Liver cancer

Your doctor will closely monitor you for complications of cirrhosis and prescribe specific medications for each complication as needed.

There’s a lot to think about when living with PBC. You may need regular checkups and tests to see how your PBC is doing and to watch for other health problems. Staying in touch with your healthcare team can help you find a treatment plan that’s right for you.

Find Your Team

On myPBCteam, the social network for people with primary biliary cholangitis and their loved ones, members come together to ask questions, give advice, and share their stories with others who understand life with primary biliary cholangitis.

Have you been diagnosed with PBC? What treatments have you tried, and did you have any side effects? Share your experiences in the comments below, or start a conversation by posting on your Activities page.