Primary biliary cholangitis (PBC) is a chronic and progressive liver disease that causes liver damage. You may already know that your liver is a unique organ that can regenerate (regrow) after some tissue damage.

Although your liver is a powerful organ, liver damage from PBC can’t be cured or reversed. With treatment, you may be able to slow or delay your disease progression.

How Does PBC Cause Liver Damage?

PBC is a condition that destroys your bile ducts — the canals that connect your liver to your small intestine to deliver bile. Your liver makes bile, a liquid that helps with digestion.

Researchers believe PBC is caused by an autoimmune disease that prompts your immune system to attack healthy cells in your bile ducts. It’s not clear why some people develop PBC, but it could be triggered by genetic and environmental factors. As your immune system continues to attack your bile ducts, they can stop working as well as they should. This makes it harder for bile to flow from your liver and into your small intestines.

PBC can cause liver damage when bile backs up in your liver. Bile contains bile acids that break down proteins and fats. If bile moves out of your bile ducts and into your liver, it can cause damage and inflammation.

Over time, the damaged liver tissue is replaced by scar tissue in a process called fibrosis. In the later stages of PBC, more and more scar tissue replaces healthy tissue in your liver. More scar tissue means that your liver doesn’t work as well as it should — a condition called cirrhosis. Eventually, cirrhosis can lead to liver failure, which is fatal.

Not everyone with PBC will reach the stage of liver failure. However, as PBC progresses, you may experience other symptoms.

Symptoms of PBC

The most common symptoms of PBC are extreme fatigue and pruritus (itchy skin). These symptoms can occur even in the early stages of PBC.

As more scar tissue replaces healthy tissue in your liver, it can cause complications, such as portal hypertension. Portal hypertension is high blood pressure in the blood vessels that run through the liver (called the portal vein).

Portal hypertension can cause problems, such as:

• Varices — Enlarged veins in your digestive tract that can cause bleeding
• Ascites — A buildup of fluid that causes an enlarged stomach
• Edema — Swelling caused by fluid buildup (swollen feet, ankles, and legs)
• Splenomegaly — An enlarged spleen
• Mental confusion — Caused by a buildup of toxins in your blood

Some symptoms of PBC are caused by a lack of bile in your digestive system. Without bile, your body has a difficult time absorbing fat, which can lead to the following symptoms:

• High cholesterol levels in your blood
• Xanthomas — Fat deposits in your skin
• Diarrhea — May include greasy stools
• Difficulty absorbing fat-soluble vitamins — Such as vitamins A, D, E, and K
• Osteoporosis — Weak bones caused by low levels of vitamin D (which helps with calcium absorption)

Additional symptoms of PBC include:

• Dry eyes and dry mouth
• Stomach pain
• Bone and joint pain
• Jaundice (yellowing of the skin)
• Hyperpigmentation (darkening of the skin)
• Hypothyroidism (low thyroid levels)
• Weight loss

PBC Can’t Be Reversed

Your liver is the only organ in your body that can regenerate. However, when you have cirrhosis, your liver doesn’t have enough healthy cells to heal itself. Continuous damage over time caused by your immune system and bile backed up in your liver results in permanent damage that can’t be healed.

There’s no cure for PBC. However, with treatment and proper care, it may be possible to slow the progression of your disease.

Treatments To Slow the Progression of PBC

Treatment options for PBC include medications and surgery:

• Ursodeoxycholic acid (UDCA)
• Obeticholic acid (Ocaliva)
• Liver transplantation

Ursodeoxycholic Acid

The first treatment for PBC is a medication called ursodeoxycholic acid. UDCA is also known as ursodiol or by the brand names Actigall and Urso.

UDCA is a naturally occurring bile salt that can help clear the bile from your liver to help prevent liver damage and cirrhosis. UDCA can’t cure PBC, but it can slow the progression of PBC in people with early-stage disease.

Obeticholic Acid

Obeticholic acid is a newer medication approved by the U.S. Food and Drug Administration (FDA) to treat PBC alone or in combination with UDCA. Obeticholic acid is another type of bile salt that also works by clearing bile from your liver. Clinical trials have found that obeticholic acid can improve liver function and help to slow liver fibrosis.

However, increased itching is a possible side effect of obeticholic acid, which may cause some people to stop using it.

Liver Transplant

You may need a liver transplant if your PBC symptoms don’t respond to medication or if your condition worsens. A liver transplant involves surgery to replace your diseased liver with a healthy liver donated by another person.

However, even with a new liver, PBC can come back in some people. If PBC does return after you receive a liver transplant, it usually progresses more slowly.

Treatments To Manage PBC Symptoms

Although PBC treatments can help prevent further progression of the disease, they may not help with other symptoms. UDCA and obeticholic acid often don’t help with the most common symptoms — fatigue and itching. In fact, obeticholic acid can make itching worse. Additional treatments can help you manage the symptoms that bother you.

People with fatigue caused by PBC may benefit from a stimulant medication called modafinil (Provigil).

There are several options for treating itching from PBC, such as:

• Antihistamines — Diphenhydramine (Benadryl), hydroxyzine hydrochloride (Vistaril), and loratadine (Claritin)
• Cholestyramine (Questran)
• Rifampin (Rifadin)
• Sertraline hydrochloride (Zoloft)
• Naltrexone (Revia)

If you have dry eyes and mouth, artificial tears and saliva substitutes can help. Sucking on hard candy or chewing gum can also help with dry mouth symptoms.

Talk to your doctor about the best treatment for you based on your symptoms.

Lifestyle Changes for PBC

Healthy lifestyle habits can help prevent PBC complications, help improve your symptoms, and make you feel better while living with PBC.

Diet Changes

When you’re diagnosed with PBC, your doctor may recommend changes to your diet, including:

• Reducing the amount of sodium (table salt) in your diet — To reduce fluid buildup from edema or ascites
• Drinking plenty of water — To improve dry eyes and mouth and improve fatigue
• Reducing the amount of fat in your diet — To avoid diarrhea and lower your cholesterol levels
• Eating a balanced diet — To ensure you get the appropriate nutrients you need
• Avoiding certain foods and drinks that can harm your liver — Talk to your doctor about what to avoid with PBC.

Physical Activity

When you have PBC, you have an increased risk of osteoporosis. To help prevent bone loss, your doctor may recommend regular exercise, such as walking and weight lifting, most days of the week.

Physical activity can also improve your overall health and prevent other diseases, such as heart disease, diabetes, and some types of cancer.

Talk With Others Who Understand

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

How do you prevent complications of PBC? What lifestyle changes have you made since your diagnosis of PBC? Share your experience in the comments below, or start a conversation by posting on your Activities page.