What is Primary Biliary Cholangitis

Primary biliary cholangitis is a long-term (chronic) liver disease. Your liver is important for functions such as absorbing nutrients from food, removing waste from the body, and making proteins and substances that help your blood clot.

Primary biliary cholangitis destroys the tube-like structures (bile ducts) in the liver that produce the digestive fluid called bile. Bile is necessary for absorbing fats, cholesterol, and fat-soluble vitamins. As bile ducts are destroyed, bile backs up in your liver and causes liver damage. It can lead to scarring of the liver (cirrhosis).

What are the causes?

The exact cause of this condition is not known. It may be an autoimmune disease. If you have an autoimmune disease, your body’s defense system (immune system) mistakenly attacks normal cells. In primary biliary cholangitis, the immune system attacks the bile ducts.

What increases the risk?

You are more likely to develop this condition if:

• You have a family history of the disease. If you inherited the gene for the disease, something might trigger the gene to become active. Possible triggers include:
  • Cigarette smoke.
  • Exposure to toxic chemicals.
  • Certain infections.
• You are a woman.
• You are 30–65 years old.

What are the signs or symptoms?

In the early stages of the disease, you might not show any signs or symptoms. The earliest symptoms are:

• Fatigue.
• Very itchy skin.
• Dry eyes or mouth.
• Pain in the abdomen, under your right rib.
• Slight yellow appearance in the whites of your eyes or in your skin (jaundice).

Later signs and symptoms develop as problems related to liver damage occur. These can include:

• Swelling of the feet and legs (edema).
• Swelling of the abdomen (ascites).
• Mental confusion and impaired mental functioning (hepatic encephalopathy).
• Darkening and increased jaundice of the skin.
• Fatty bowel movements.
• Worsening right-sided abdominal pain.
• Vomiting blood.

How is this diagnosed?

Your health care provider may suspect primary biliary cholangitis if you have abnormal results in a blood test for liver function. This test is often done as part of a routine physical exam. Your health care provider may also check to see if:

• Your liver is enlarged.
• Your spleen is enlarged.
• You have jaundice.
• You have swelling in your legs or abdomen.

Tests may also be done to confirm the diagnosis. These may include:

• A blood test. This looks for a specific type of immune system protein (antimitochondrial antibodies, or AMA).
• Imaging studies. An ultrasound is used to create pictures of your liver.
• Liver biopsy. A piece of tissue from your liver is checked under a microscope.

How is this treated?

For early treatment of this condition, your health care provider will prescribe a bile substitute medicine (ursodiol). You will likely need to take this medicine for the rest of your life. Also, you should avoid:

• Alcohol.
• Hepatotoxic medicines. These are medicines that are damaging to the liver when taken too often or in excessive amounts. These can include:
  • Over-the-counter and prescription medicines.
  • Herbal treatments.
  • Vitamins.
  • Dietary supplements.

As primary biliary cholangitis progresses, you may need other treatments, such as:

• Antihistamines to relieve itching.
• Artificial tears and saliva to relieve dry eyes and mouth.
• Medicines that lower blood pressure and reduce swelling (diuretics).
• A laxative (lactulose) to remove toxic substances that cause hepatic encephalopathy.
• A liver transplant if primary biliary cholangitis develops into cirrhosis.

The earlier you start treatment, the more likely it is that your condition will be slowed down.

Follow these instructions at home:

Lifestyle

• Work with a dietitian to create a meal plan that gives you the right amount of protein and nutrition with less salt.
• Get regular exercise. Ask your health care provider what the right amount of exercise is for you.
• Do not use any products that contain nicotine or tobacco, such as cigarettes and e-cigarettes. If you need help quitting, ask your health care provider.
• Do not drink alcohol.
• Do not eat raw shellfish.

General instructions

• Learn as much as you can about your disease, and work closely with your health care providers.
• You may need to have regular blood tests to monitor your liver function.
• Take over-the-counter and prescription medicines only as told by your health care provider.
• Drink enough fluid to keep your urine pale yellow.

Contact a health care provider if:

• Your symptoms are gradually getting worse.

Get help right away if:

• You vomit blood.
• Your itching suddenly worsens or stops.
• You develop new jaundice.
• You become confused or pass out.
• You develop sudden new swelling in your legs or abdomen.

Summary

• Primary biliary cholangitis is a chronic liver disease that destroys the bile ducts in the liver. It can lead to cirrhosis. It may be an autoimmune disease.
• You are more likely to develop this disease if others in your family have it, if you are female, and if you are 30–65 years old.
• Early symptoms include fatigue, itching, and pain in the abdomen. Later symptoms include ascites, edema, worsening jaundice, and hepatic encephalopathy.
• Diagnosis of primary biliary cholangitis may involve blood tests, imaging tests such as ultrasound, and liver biopsy.
• Medicines are available to treat this condition. You may also be required to limit salt, avoid hepatotoxic medicines, and stop drinking alcohol.