Everything You Need to Know About Primary Biliary Cholangitis

Medically Reviewed By Saurabh Sethi, M.D., MPH
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Primary biliary cholangitis is a chronic autoimmune disease in which the body attacks healthy bile duct cells. This causes inflammation and eventual death of the small bile ducts in the liver. When the bile ducts are gone, bile builds up and causes liver damage. This damage leads to liver scarring, cirrhosis, and liver failure. Primary biliary cholangitis, previously known as primary biliary cirrhosis, is more common in middle-aged women. A diagnosis typically occurs between ages 30 and 60. There is a strong prevalence of the disease among first-degree relatives and identical twins.

Read on to learn more about the stages, causes, symptoms, and treatment for primary biliary cholangitis.

Sex and gender exist on spectrums. This article will use the terms “men” and “women” to refer to a person’s sex assigned at birth.

What are the stages of primary biliary cholangitis?

A woman looking tired
Julia Potato/Stocksy United

There are four stages of primary biliary cholangitis.

Stage Symptoms
1The bile ducts show signs of damage. The portal vein, which is the major vein leading to the liver, becomes inflamed.
2The areas surrounding the portal vein become thickened or scarred.
3Scar tissue builds up, and inflammation spreads.
4Cirrhosis sets in.

What are the causes of primary biliary cholangitis?

The exact cause of primary biliary cholangitis is unknown. Immunological, autoimmune, genetic, and environmental factors may play a role. People with primary biliary cholangitis usually have decreased levels of T cells and abnormalities in T cell function. T cells help defend the body from yeast, fungi, viruses, and bacteria.

About 95% of people with primary biliary cholangitis produce antibodies that act on the body’s mitochondria. Mitochondria are found in the body’s cells, and they contain the blueprints for making energy.

Environmental factors may trigger primary biliary cholangitis in people with certain genes. These triggers can include infections, cigarette smoke, or exposure to certain chemicals.

What are the symptoms of primary biliary cholangitis?

Some of the most common symptoms of primary biliary cholangitis are itching, fatigue, and jaundice. However, people may also experience:

As the condition worsens, symptoms may include:

  • darkening of skin color
  • fatty deposits called xanthomas that appear as yellow bumps on the skin
  • other symptoms of cirrhosis, such as swelling and weight loss
  • enlarged spleen
  • enlarged liver

Some people with primary biliary cholangitis may not display symptoms.

How do doctors diagnose primary biliary cholangitis?

To diagnose primary biliary cholangitis, your doctor will ask about your medical and family history. They may ask you questions such as:

  • Do you have a family history of autoimmune diseases?
  • Does one of your immediate family members have primary biliary cholangitis?
  • Do you have a history of infections?
  • Are you frequently exposed to chemicals?

Your doctor will also perform a physical examination to check for:

  • yellowing of the eyes or skin
  • enlargement of the liver or spleen
  • abdominal tenderness or pain, specifically in the upper right side of your abdomen

Blood tests can also help doctors confirm a diagnosis, including:

  • Anti-mitochondrial antibodies (AMA): These antibodies appear in the blood of 95% of people with this condition.
  • Liver tests: These tests can show whether you have atypical liver enzyme levels, such as alkaline phosphatase. It can block the bile ducts.
  • Cholesterol: People with primary biliary cholangitis may have elevated cholesterol levels.

Your doctor may also perform imaging tests such as X-rays or ultrasounds to help diagnose primary biliary cholangitis. These tests can rule out any other possible causes of bile duct damage. To determine the stage of the disease, your doctor may take a small sample of your liver.

What are the treatments for primary biliary cholangitis?

A common treatment for primary biliary cholangitis is a medication called ursodiol. It is a bile salt that slows the progression of the disease. When started early, it can help prevent the development of liver cirrhosis. Furthermore, people who receive early treatment can live longer without needing a liver transplant.

In 2016, the U.S. Food and Drug Administration (FDA) approved a drug called Ocaliva for people who cannot tolerate or do not respond well to ursodiol. The National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK) notes that people with cirrhosis should not use Ocaliva because it can worsen symptoms.

Doctors also treat the symptoms of primary biliary cholangitis with other medications, supplements, and lifestyle changes. If you have elevated cholesterol levels, your doctor may recommend you take a medication from a class called statins. They may also recommend eating a healthy diet and exercising.

Dietary supplements of vitamins A, D, E, and K can be beneficial if your body has difficulty absorbing fat-soluble vitamins. Your doctor may recommend regular eye and dental checkups if you have dry eyes or mouth.

A liver transplant might be necessary in severe cases when primary biliary cholangitis leads to liver failure. Doctors will only consider this if all other treatment options have failed or are not feasible.

What are some potential complications of primary biliary cholangitis?

Complications can occur when primary biliary cholangitis reaches advanced stages. These complications may include:

What are the risk factors for primary biliary cholangitis?

Some factors increase the risk of developing primary biliary cholangitis. However, not all people with risk factors develop the condition. Common risk factors include:

  • being female
  • being middle-aged
  • being white
  • having a parent or sibling with primary biliary cholangitis
  • having an autoimmune disease
  • smoking cigarettes
  • being frequently exposed to chemicals

What is the outlook for people with primary biliary cholangitis?

Primary biliary cholangitis is not curable. However, the outlook for people with the condition is better with prompt treatment.

Treatment with bile salt therapy can improve life expectancy in individuals with primary biliary cholangitis. It can also delay or prevent the need for a liver transplant. The disease progresses slowly. People who show no symptoms at the time of diagnosis can remain symptom-free for years.

Primary biliary cholangitis can reoccur after a liver transplant.


Primary biliary cholangitis is an autoimmune disease that causes liver damage. Experts believe it occurs when the body attacks its own healthy bile duct cells. It is more common in middle-aged women and those with a first-degree relative with the disease.

Common symptoms of primary biliary cholangitis include itching, fatigue, and jaundice. Doctors typically diagnose the condition through physical examinations, blood tests, and imaging tests. Treatment can include medications to slow the progression of the disease, diet and lifestyle changes, or vitamin supplements. In some cases, a liver transplant may be necessary.

Talk with your doctor about your treatment options for primary biliary cholangitis. With treatment, people with the disease can live longer, healthier lives.

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Medical Reviewer: Saurabh Sethi, M.D., MPH
Last Review Date: 2022 Jun 21
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