What Is Primary Biliary Cholangitis?

Primary biliary cholangitis is a rare liver disease. It blocks and destroys the tubes that go from your liver to your gallbladder and small intestine. Doctors used to call it “primary biliary cirrhosis.”

“Biliary” means bile. That’s a digestive fluid that helps your body get rid of extra fats, like too much cholesterol, and other things your body doesn’t need.

“Cholangitis” is the inflammation that happens because something, such as gallstones, block the tubes that go from your liver to your small intestine.

If you have this condition, bile may not be able to move out of the liver. So the things that bile gets rid of can build up or move into other parts of the body. Than can scar and damage organs. It can get worse over time.

Who Gets It?

Only about 4 to 15 out of 1 million people get this condition. Most often, it affects women between ages 30 and 65.

Sometimes it runs in families. You could be more likely to get it if you have a mom, dad, brother, or sister -- especially an identical twin -- with the disorder.

Causes

Doctors don’t know what causes primary biliary cholangitis. Researchers are studying how genes, changes to the immune system, and other things may play a role.

People with this condition have very low levels of certain white blood cells, called circulating T cells, which help your body fight infections. These cells may work differently in people with primary biliary cholangitis, too. It’s not yet clear how or if these differences trigger the liver disorder or its symptoms.

Another theory is that the disease is an immune system problem.

Symptoms

You might not have symptoms at first. In more than half of people who have this condition (up to 60%), doctors find it unexpectedly when someone gets blood tests to check how well their liver works.

If you do have symptoms, the first and most common ones are:

  • Itching due to a buildup of toxins in the body
  • Darkened skin
  • Feeling tired all the time (fatigue)
  • Dry eyes and mouth
  • Joint pain
  • Upper right side belly pain
  • Weight loss

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You may also have yellow skin and whites of the eyes (jaundice) due to a buildup of bile in the liver.

Over time, your liver tissue can scar and harden. Other complications can start as the liver stops working the way it should and the bile ducts become blocked. Gallstones and bile duct stones can form, causing pain and infections.

Other complications of PBC include:

High blood pressure in the liver vein (portal hypertension). This can lead to fluid buildup in your feet, ankles, legs, and abdomen. Your spleen may swell.

Hepatic encephalopathy. Toxins that the liver usually removes start to build up in the organ and eventually the brain. This can cause confusion, personality changes, and even coma.

Swollen blood vessel (varices) in the esophagus and stomach. These can burst and cause serious, life-threatening bleeding.

Vitamin and metabolic deficiencies. When the bile ducts become blocked, the liver can no longer filter substances, such as cholesterol and other fats, as it normally would. This changes the way the body breaks down fats and vitamins A, D, E, and K. As a result, your body can’t use or nutrients the way it once did. Osteoporosis can happen when your body has problems using vitamin D and calcium.

Other autoimmune disorders. It's not unusual to find other autoimmune diseases diagnosed in patients with PBC. Those may inclusde such conditions as Sjögren syndrome and autoimmune thyroid disease. Rheumatoid arthritis is common in 40% to 60% of people with PBC.

Steatorrhea. When bile can’t get to the small intestine, your body cannot absorb fat properly and you get this condition. Fat then builds up in your stools, creating loose, greasy, and foul-smelling bowel movements.

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Diagnosis

Your doctor will give you a physical exam and ask questions about your family and medical history. You may also get these blood tests:

  • Alkaline phosphatase, a test for increased liver enzymes
  • AMA, a test to see if your body is making antibodies that attack your cells’ energy powerhouses, or mitochondria. Your doctor may call these “antimitochondrial antibodies.”

You could also need imaging tests, such as an ultrasound, MRI, or X-ray of your bile ducts.

Your doctor may also want to do a liver biopsy, where she takes a small sample of tissue to test in a lab.

You have primary biliary cholangitis if you have at least two of the following:

  • High levels of alkaline phosphatase
  • Antimitochondrial antibodies
  • Signs of the disease on a liver biopsy

Treatment

The main treatment for PBC is a drug called ursodiol. It’s a natural form of bile that can help your liver work better and help you live longer. You take it as a pill, unless you get a new liver in a transplant. There are few reported side effects from the drug.

If you can’t take ursodiol, or it doesn’t work well enough for you, your doctor may prescribe obeticholic acid (Ocaliva) along with, or instead of, ursodiol.

Your doctor may prescribe other treatments to ease symptoms and may include:

  • Medications to relieve itching, such as cholestyramine or colestipol
  • Drugs to lower blood pressure in the veins
  • Diuretics, or water pills, to remove extra fluid from your body
  • Plasmapheresis, a procedure to remove unwanted substances from the blood
  • Vitamin supplements to treat vitamin K, A, D, calcium, and iron deficiencies
  • Low-fat diet with medium-chain triglycerides to boost calorie intake

If you develop liver failure or complications that can no longer be effectively treated, your doctor may recommend that you get on the list for a liver transplant.

Tips for Living With Primary Biliary Cholangitis

If you have this condition, you should avoid drinking alcohol and never use illegal drugs. These things can further damage your liver.

Tell your doctor before you use any type of over-the-counter medications, including and especially herbs and supplements. Many substances can affect how your liver works and may interfere with your treatment and outcome.

Liver cancer is a concern in people who develop scarring of the liver, called cirrhosis. Talk to your doctor about what tests you should get. You may need to be checked for the disease every 6 to 12 months.

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Resources

You can find more information on primary biliary cholangitis from:

  • American Liver Foundation
  • The National Institute of Diabetes and Digestive and Kidney Diseases
  • National Organization for Rare Disorders
WebMD Medical Reference Reviewed by Sabrina Felson, MD on August 07, 2018

Sources

SOURCES:

American Liver Foundation: “Primary Biliary Cholangitis (PBC, Primary Biliary Cirrhosis).”

National Organization for Rare Disorders: “Primary Biliary Cholangitis.” 

Merck Manual: “Overview of Gallbladder and Bile Duct Disorders.”

The National Institute of Diabetes and Digestive and Kidney Diseases: “Primary Biliary Cirrhosis.”

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