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Primary Biliary Cholangitis (Primary Biliary Cirrhosis)

Definition & Facts

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What is primary biliary cholangitis?

Primary biliary cholangitis is a chronic disease in which the small bile ducts in the liver become inflamed and are eventually destroyed. When there are no bile ducts, bile builds up and causes liver damage. Over time, this damage can lead to liver scarring, cirrhosis, and eventually liver failure. Primary biliary cholangitis is believed to be an autoimmune disease in which a person’s own immune system becomes overactive and attacks normal, healthy bile duct cells.

Does primary biliary cholangitis have another name?

Doctors and patients often use the abbreviation PBC for primary biliary cholangitis. The disease used to be called primary biliary cirrhosis.

How common is primary biliary cholangitis?

Researchers estimated that in 2014 about 58 out of every 100,000 U.S. women and about 15 out of every 100,000 U.S. men had primary biliary cholangitis.1

Who is more likely to develop primary biliary cholangitis?

Primary biliary cholangitis is more common in

  • women than in men.
  • people who are middle aged. The average age at diagnosis is 60.2
  • people who are white compared with other racial or ethnic groups.
  • people who have a parent or sibling—particularly an identical twin—with primary biliary cholangitis.
A health care professional talking with a patient who is a woman.Primary biliary cholangitis is more common in women than in men.

What other health problems do people with primary biliary cholangitis have?

People with primary biliary cholangitis may have other autoimmune diseases, including

Women with primary biliary cholangitis may also have frequent urinary tract infections.

What are the complications of primary biliary cholangitis?

Common complications of primary biliary cholangitis include

These common complications can be prevented and treated.

Liver complications

Primary biliary cholangitis can also damage the liver, leading to liver complications.

Cirrhosis. In cirrhosis, scar tissue replaces healthy liver tissue and prevents your liver from working normally. As cirrhosis gets worse, the liver begins to fail.

Portal hypertension. Portal hypertension most often occurs when scar tissue in a liver with cirrhosis partly blocks and slows the normal flow of blood, which causes high blood pressure in the portal vein. However, people with primary biliary cholangitis may develop portal hypertension before they develop cirrhosis. When portal hypertension reaches a certain level, it can cause additional complications, such as

  • swelling in your legs, ankles, or feet, called edema
  • buildup of fluid in your abdomen, called ascites
  • enlarged veins—called varices—in your esophagus, stomach, or intestines, which can lead to internal bleeding if the veins burst
  • confusion or difficulties thinking caused by the buildup of toxins in your brain, called hepatic encephalopathy

Liver failure. Cirrhosis may eventually lead to liver failure, also called end-stage liver disease. With liver failure, your liver is badly damaged and stops working. People with liver failure may require a liver transplant.

Liver cancer. Research suggests that people with cirrhosis caused by primary biliary cholangitis, as well as men with primary biliary cholangitis have an increased risk for liver cancer.

References


Symptoms & Causes

What are the symptoms of primary biliary cholangitis?

The most common symptoms of primary biliary cholangitis are

  • feeling tired
  • itchy skin

Other symptoms may include

As the disease gets worse, symptoms may include

Many people have no symptoms when they are first diagnosed with primary biliary cholangitis. Doctors diagnose about 60% of people with primary biliary cholangitis before symptoms begin.3 People with primary biliary cholangitis and no symptoms are identified through blood tests. Some people do not have symptoms for years after they have been diagnosed with primary biliary cholangitis.

Tired woman sitting in front of a laptop.The most common symptoms of primary biliary cholangitis are feeling tired and having itchy skin.

What causes primary biliary cholangitis?

Experts aren’t sure what causes primary biliary cholangitis. Studies suggest that certain genes make some people more likely to develop the disease. In people with these genes, factors in the environment may trigger an autoimmune reaction that causes their immune system to attack the small bile ducts in the liver, causing primary biliary cholangitis.

Possible environmental triggers include

  • infections
  • cigarette smoking
  • exposure to certain chemicals

References


Diagnosis

How do doctors diagnose primary biliary cholangitis?

Doctors diagnose primary biliary cholangitis based on your medical and family history, a physical exam, and the results of medical tests.

Medical and family history

Your doctor will ask you about your symptoms. He or she will also ask

  • whether you have a history of certain autoimmune diseases
  • whether one of your parents or siblings has been diagnosed with primary biliary cholangitis
  • whether you have a history of infections and exposure to certain chemicals

Physical exam

Your doctor will examine your body, use a stethoscope to listen to sounds in your abdomen, and tap or press on specific areas of your abdomen. He or she will

  • look for yellowing of the whites of your eyes and your skin
  • check to see if your liver and spleen are larger than they should be
  • check for abdominal tenderness or pain, particularly in the upper-right side of your abdomen

What tests do doctors use to diagnose primary biliary cholangitis?

Blood tests

For a blood test, a health care professional will take a blood sample from you and send the sample to a lab. Your doctor may recommend the following blood tests.

Anti-mitochondrial antibodies (AMA). Anti-mitochondrial antibodies are found in the blood of about 95% of people with primary biliary cholangitis.4

Liver tests. Liver tests can show abnormal liver enzyme levels, which may be a sign of damage in your liver or biliary tract. Higher-than-normal levels of the liver enzyme alkaline phosphatase occur in people with diseases that destroy or block the bile ducts, such as primary biliary cholangitis.

Your doctor may diagnose primary biliary cholangitis if you have anti-mitochondrial antibodies and higher-than-normal levels of alkaline phosphatase in your blood, even if you have no other signs or symptoms of the disease.

Cholesterol. People with primary biliary cholangitis may have higher-than-normal cholesterol levels.

Close-up of a health care professional preparing to take a blood sample from a patient’s arm.Your doctor may recommend blood tests to diagnose primary biliary cholangitis.

Imaging tests

Your doctor may use imaging tests such as x-rays and ultrasounds to help diagnose primary biliary cholangitis by ruling out other causes of bile duct damage, such as gallstones, bile duct strictures, and tumors.

Liver biopsy

During a liver biopsy, a doctor will take small pieces of tissue from the liver. A pathologist will examine the tissue with a microscope. Your doctor may perform a liver biopsy to

  • rule out other diseases that may be causing your symptoms
  • confirm the diagnosis of primary biliary cholangitis
  • determine whether the disease is advanced—as shown by the amount of liver scarring or cirrhosis—or is very active

References


Treatment

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How do doctors treat primary biliary cholangitis?

Doctors treat primary biliary cholangitis with medicines. Your doctor may prescribe ursodiol. Although ursodiol does not cure primary biliary cholangitis, it can slow the progression of liver damage. People who respond to ursodiol early in the course of primary biliary cholangitis can live longer without needing a liver transplant.

If you do not respond to ursodiol, your doctor may prescribe obeticholic acid. Although obeticholic acid may improve blood test results, it may worsen symptoms. Obeticholic acid has not been shown to improve the course of illness or prevent cirrhosis. People with cirrhosis should not use obeticholic acid because it can cause worsening of disease.

An open hand holding two medicine capsules.Doctors treat primary biliary cholangitis with medicines.

How do doctors treat the symptoms of primary biliary cholangitis?

Your doctor may recommend over-the-counter medicines or prescribe medicines to treat symptoms of primary biliary cholangitis, such as itchy skin.

How do doctors treat the complications of primary biliary cholangitis?

Doctors may treat common complications of primary biliary cholangitis with medicines, dietary supplements, or changes in your diet and lifestyle.

  • If you have higher-than-normal blood cholesterol levels, your doctor may prescribe medicines called statins and recommend lifestyle changes.
  • For osteoporosis, your doctor may prescribe medicines that slow or stop bone loss and improve bone density. Your doctor may recommend dietary supplements of calcium and vitamin D.
  • For low levels of fat-soluble vitamins, your doctor may recommend dietary supplements of vitamins A, D, E, and K. Follow your doctor’s instructions on the type and amount of vitamins you should take.
  • If you have dry eyes and dry mouth due to Sjögren’s syndrome, you should have regular eye and dental examinations.

Liver complications

Doctors may recommend additional treatments for liver complications of primary biliary cholangitis.

Cirrhosis or portal hypertension. If primary biliary cholangitis leads to cirrhosis or portal hypertension, doctors can treat the health problems related to these conditions with medicines, minor medical procedures, and surgery.

Liver failure. If cirrhosis leads to liver failure, you may need a liver transplant.

Liver cancer. If you have cirrhosis or other risk factors, your doctor may suggest blood tests and an ultrasound or another type of imaging test to check for liver cancer. Finding cancer at an early stage improves the chance of curing the cancer.

When do doctors consider a liver transplant for primary biliary cholangitis?

Your doctor will consider a liver transplant when your primary biliary cholangitis leads to liver failure. Doctors consider liver transplants only after they have ruled out all other treatment options. Talk with your doctor to find out whether a liver transplant is right for you.

What can I do to help prevent further liver damage?

If you have primary biliary cholangitis, you can take steps to help prevent further liver damage. For example, you can

  • carefully follow your doctor’s instructions and take your medicines and dietary supplements as directed.
  • quit smoking.
  • avoid drinking alcohol. If you have cirrhosis, completely stop drinking alcohol.
  • have regular checkups, as recommended by your doctor.
  • talk with your doctor before taking
  • try to reach and stay at a healthy body weight.


Eating, Diet, & Nutrition

What should I eat if I have primary biliary cholangitis?

You should eat a healthy, well-balanced diet. Good nutrition is important in all stages of primary biliary cholangitis to help your liver work properly and manage complications.

Your doctor can recommend a healthy eating plan that is well-balanced and provides enough calories and nutrients. Your doctor may recommend that you eat foods high in calcium and vitamin D or take dietary supplements of these nutrients to help prevent osteoporosis. If you have low levels of fat-soluble vitamins A, D, E, or K, your doctor may also recommend supplements of these vitamins. Follow your doctor’s instructions on the type and amount of vitamins you should take.

A husband and wife cutting vegetables in a kitchen.Good nutrition is important in all stages of primary biliary cholangitis.

What foods should I avoid eating if I have primary biliary cholangitis?

You should avoid eating raw or undercooked shellfish, fish, meat, and unpasteurized milk. Bacteria or viruses from these foods may cause severe infections in people with liver disease.

If you have primary biliary cholangitis, your doctor will recommend that you quit smoking and stop drinking alcohol or, at least, limit your intake. If you have primary biliary cholangitis and cirrhosis, you should completely stop drinking alcohol.


Clinical Trials

The NIDDK conducts and supports clinical trials in many diseases and conditions, including liver diseases. The trials look to find new ways to prevent, detect, or treat disease and improve quality of life.

What are clinical trials for primary biliary cholangitis?

Clinical trials—and other types of clinical studies —are part of medical research and involve people like you. When you volunteer to take part in a clinical study, you help doctors and researchers learn more about disease and improve health care for people in the future.

Researchers are conducting clinical studies to better understand liver diseases, such as primary biliary cholangitis. Find out if clinical studies are right for you.

Watch a video of NIDDK Director Dr. Griffin P. Rodgers explaining the importance of participating in clinical trials.

What clinical studies for primary biliary cholangitis are looking for participants?

You can find clinical studies on primary biliary cholangitis at www.ClinicalTrials.gov. In addition to searching for federally funded studies, you can expand or narrow your search to include clinical studies from industry, universities, and individuals; however, the National Institutes of Health does not review these studies and cannot ensure they are safe. Always talk with your health care provider before you participate in a clinical study.


This content is provided as a service of the National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK), part of the National Institutes of Health. The NIDDK translates and disseminates research findings to increase knowledge and understanding about health and disease among patients, health professionals, and the public. Content produced by the NIDDK is carefully reviewed by NIDDK scientists and other experts.

The NIDDK would like to thank:
John Moore Vierling, M.D., Baylor College of Medicine