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.
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
autoimmune thyroid diseases—conditions in which the immune system attacks the thyroid gland
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
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.
Symptoms & Causes
What are the symptoms of primary biliary cholangitis?
The most common symptoms of primary biliary cholangitis are
Other symptoms may include
discomfort or pain in the upper-right side of your abdomen
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.
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
exposure to certain chemicals
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
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.
Your doctor may recommend blood tests to diagnose primary biliary cholangitis.
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.
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
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.
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?
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.
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.
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.
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.
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.
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