• Primary biliary cholangitis (PBC) is a liver disease that gradually worsens, so it’s important to slow down its progression and prevent long-term liver damage.
• There’s currently no cure for PBC.
• Managing PBC involves addressing symptoms like itchy skin, dry mouth, dry eyes, and potential complications with medications, surgery, supplements, and lifestyle adjustments.

Primary biliary cholangitis — previously known as primary biliary cirrhosis — is a chronic and progressive liver disease. Once PBC begins, it’ll continue to get worse over time. Unfortunately, there’s currently no definite cure for PBC. Treatment focuses on slowing down the disease and preventing permanent liver damage.

Doctors use medications to treat PBC and to help manage symptoms. If liver failure occurs in late-stage PBC, a liver transplant may be necessary. After a PBC diagnosis, it’s important to work with your doctors to develop a treatment plan.

Treatment Options for PBC

Doctors and researchers believe that PBC is an autoimmune disease that causes your immune system to attack the small bile ducts in your liver. 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’s normally secreted through the bile ducts into the small intestine. It’s responsible for breaking down fatty acids and helping your body absorb vitamins and cholesterol. Damaged bile ducts can’t carry bile to your digestive system, causing the fluid to build up in your liver. Without enough bile, your body can’t absorb the necessary vitamins. Eventually, the scar tissue interferes with your liver function and can lead to cirrhosis.

Your doctor may prescribe medications to help move bile through your liver. Other treatments target inflammation and scar tissue formation to slow PBC progression.

Ursodeoxycholic Acid

After a PBC diagnosis, the first medications that doctors typically prescribe are bile acids. Your body naturally makes bile acids to help dissolve gallstones and absorb cholesterol.

Doctors typically first prescribe the bile acid ursodeoxycholic acid (UDCA) — also known as ursodiol (sold as Urso and Actigall). The U.S. Food and Drug Administration (FDA) approved UDCA for treating PBC in 2004. This medication helps move bile through the liver into the small intestine.

According to the American Liver Foundation, UDCA is effective in more than 50 percent of people with PBC. It’s important to start taking UDCA in the early stages of PBC because it may help improve your liver function and slow disease progression. While UDCA likely won’t help treat fatigue or skin itching, it can help reduce liver scarring and may prevent the need for (or delay) a liver transplant.

However, up to 40 percent of people don’t respond to UDCA. This means that treatment doesn’t help lower their alkaline phosphatase (ALP) or bilirubin levels. These biomarkers let doctors know UDCA isn’t working. Up to 10 percent of people with PBC can’t tolerate UDCA.

Mayo Clinic notes that UDCA side effects can include hair loss, weight gain, and diarrhea.

Obeticholic Acid

Another treatment for PBC is obeticholic acid (Ocaliva). The FDA approved obeticholic acid in 2016. It may be used:

• In combination with UDCA to treat PBC in people who don’t respond to UDCA treatment alone
• By itself as an alternative treatment option for people who can’t tolerate UDCA

Like UDCA, obeticholic acid also helps bile flow from the liver to the digestive system and reduces bile acid production. Studies show that treatment with UDCA and obeticholic acid can help slow liver fibrosis while improving liver function.

Side effects of obeticholic acid include pruritus (itchy skin), fatigue, headaches, and swollen nasal passages. If you’re already experiencing itchy skin from PBC, obeticholic acid may make it worse.

The FDA also issued a black box warning stating that obeticholic acid may cause liver damage in people with cirrhosis. If you have liver cirrhosis along with PBC, your doctor will likely prescribe a different medication.

Fenofibrate

Fibrates are a class of medications that treat high cholesterol. Specifically, fibrates help control cholesterol production in the liver. If you’ve tried UDCA, and it doesn’t work well, your doctor may suggest trying fenofibrate (sold under several brand names, including Antara, Fenoglide, TriCor, Triglide, and Trilipix).

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 treat skin itching in PBC.

Side effects of fibrates include:

• Diarrhea or constipation
• Stomach pain
• Leg cramps
• Headaches
• Dizziness

Budesonide

Corticosteroids like budesonide help control inflammation in the body. Doctors may prescribe budesonide with UDCA to treat PBC. However, corticosteroids are also associated with unwanted side effects like weight gain and osteoporosis (brittle, weak bones). For now, larger and more long-term studies are needed to find out whether budesonide is safe and effective for treating PBC.

Liver Transplant

Because PBC is a progressive disease — meaning it continues to get worse over time — medications may eventually stop working, and cirrhosis will lead to liver failure. 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.

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.

Treatments for Itchy Skin

Intense, itchy skin is a common and uncomfortable symptom of PBC. Over-the-counter (OTC) 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. You can find these medications at your local drugstore or grocery store.

Your doctor may also prescribe treatments like:

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

Treatments for Dry Mouth and Dry Eyes

Many people with PBC also have other autoimmune diseases at the same time (known as comorbid conditions or comorbidities). People with both PBC and Sjögren’s syndrome may experience dry mouth and eyes.

Saliva substitutes like mouthwashes are available by prescription or in stores. Mayo Clinic also suggests chewing gum or sucking on hard candy to help treat dry mouth. Artificial tears or eyedrops are also available from your doctor or OTC. If you’re living with PBC and Sjögren’s syndrome, be sure to have regular dental and eye exams.

Treating Complications of PBC

Liver damage from PBC also leads to other health complications, like vitamin deficiencies, fluid buildup, and bone problems. 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.

Low vitamin D levels can also lead to osteoporosis. Vitamin D and calcium supplements help support healthy bones. Your doctor may also suggest light exercise like walking to improve your bone density.

Low vitamin K levels can lead to problems with blood clotting in PBC, making bleeding more likely. In cirrhosis, a lack of vitamin K can also worsen clotting issues and contribute to the risk of severe bleeding. Your doctor may prescribe vitamin K supplements.

High blood pressure in the liver veins — known as portal hypertension — can cause fluid buildup throughout your body. If you notice any new edema (swelling) in your feet or ankles, talk to your doctor. You may also have ascites — fluid buildup in your abdomen. Edema and ascites can be treated with a low-salt diet. If changing your diet doesn’t help, your doctor can prescribe medications like diuretics.

Managing PBC is a complex process with various treatment options. You and your doctor should carefully consider the pros and cons of each treatment, choosing the one that best fits your needs and medical history. Understanding the available treatments and their possible side effects allows you to work with your health care teams to make informed decisions, ultimately improving your quality of life and overall health.

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 tips 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.