Primary Biliary Cholangitis

Key points

• Primary Biliary Cholangitis (PBC) is a rare condition that affects the small bile ducts (the network of small tubes that carry bile in the liver)
• Over time, the bile ducts can become inflamed and scarred
• PBC may eventually lead to long-term liver damage and liver failure
• If treatment is started early, the outcome can be excellent
• Without treatment, the condition is likely to get worse
• Some people have disease that responds poorly to treatment and may need a liver transplant

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What is PBC?

Primary Biliary Cholangitis (PBC) is thought to be an autoimmune disease. That means the body’s own immune system attacks healthy cells and tissue.

If you have PBC, your immune system starts to attack the small bile ducts. The ducts get inflamed and blocked. Eventually, they are destroyed. If this happens, bile builds up and causes liver damage.

PBC is a chronic disease (it lasts for a long time) and without treatment it gradually gets worse. Over 15 to 20 years, someone with PBC can slowly develop liver failure.

Although PBC is a very rare disease, the number of people who have it is increasing in Australia. Almost all of them (95%) are in women aged 35 to 70. It’s most common in middle age, between 45 and 65.

PBC used be called Primary Biliary Cirrhosis.

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What are the symptoms of PBC?

PBC often doesn’t cause any symptoms. People only find out they have PBC because they have a blood test for something else.

If you do have symptoms, they usually start with feeling very tired (fatigue) and having itchy skin.

As the PBC gets worse, other symptoms may start. These include:

• Dry eyes and mouth
• Small yellow or white bumps around the eyes, eyelids, palms, soles, elbows or knees (these are deposits of cholesterol)
• Diarrhoea or oily poos because the body can’t digest fat properly
• Swollen feet and ankles
• Swollen belly due to build-up of fluid
• Darkening of the skin
• Enlarged liver or spleen
• Weight loss
• Yellowing of the skin and eyes (jaundice). This is a sign the disease is quite advanced
• Pain in the upper right of the belly
• Pain in the joints, muscles or bones

PBC can also cause other problems such as:

• Liver scarring (cirrhosis)
• Osteoporosis (brittle bones)
• Portal hypertension – the portal vein is the main blood vessel that carries blood into the liver. When blood can’t flow into the liver, pressure builds up in the vein. To relieve pressure in the portal vein, the blood takes detours around the liver in other veins in the oesophagus (food pipe) or stomach. This condition can lead to swelling of the belly (ascites) as fluid leaks out of the of the liver, or swelling in other veins (varices). When veins get swollen, they can burst and bleed, which can be life threatening
• Gallstones or bile duct stones
• High cholesterol
• Underactive thyroid
• Vitamin deficiency (your body can’t absorb vitamins including A, D, E and K)
• Problems with memory and concentration

Read more about the symptoms of liver disease

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How is PBC diagnosed?

If your doctor thinks you may have PBC, they will order some blood tests to check for signs of liver disease or injury to the bile ducts. The blood will also be checked for antibodies that signal you may have PBC. These are called antimitochondrial antibodies (AMA). You will probably also have a cholesterol test.

Some people may also need an ultrasound, FibroScan or other imaging to look at the bile ducts and rule out other diseases that have similar symptoms.

If it’s still not clear what’s causing the disease, you may need a liver biopsy. This is when a small piece of tissue is removed from the liver to be examined under a microscope.

It’s recommended that people with PBC are also checked for other autoimmune diseases such as coeliac disease, thyroid disease and Sjögren’s syndrome.

Read more about liver tests

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Why did I get PBC?

We don’t fully understand what triggers the body to turn against its own cells. It’s possible that you inherit genes from your family, or that something in your life caused it to happen. Some of the triggers for PBC are thought to be:

• smoking
• exposure to some chemicals
• urinary tract infections

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How is PBC treated?

There’s no cure for PBC, but medications can be used to slow it down and prevent complications. Medicines you might be given include:

There are also treatments for some of the complications of PBC. Your doctor might give you medicine to treat high cholesterol, osteoporosis or portal hypertension. You will need regular liver function checks. If you start to develop liver cirrhosis, this will also need to be treated.

Eventually, the PBC can lead to liver failure. Having a liver transplant is usually very successful. Sometimes the PBC can come back, but it rarely leads to liver cirrhosis after a transplant.

 

Read more about liver treatments

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Living with PBC

If you’ve been diagnosed with PBC, there are a few things you can do to make yourself feel better.

• Cut down on salt. This will help with fluid retention and swelling
• Cut down on the fat in your diet, especially if you are overweight
• Drink plenty of water
• Cut out alcohol
• Don’t take any drugs that can harm the liver (including illegal drugs, alternative medicines and liver tonics)
• Do more exercise. Walking is great. Try to do some weight training as well to protect your bones
• De-stress if you can
• Stop smoking
• Look after your skin, especially if you are very itchy
• Get your teeth checked regularly, especially if you have a dry mouth

There are things you can do to manage some of the more bothersome symptoms of PBC.

Tiredness

People with PBC often feel very tired. It’s not a sign of how serious the disease is – sometimes people in the early stages feel very tired while others with more advanced disease don’t feel tired at all. To treat tiredness, your doctor will talk to you about your diet, exercise and sleep. They may also test your thyroid as thyroid disease is more common in people with PBC and can also make you tired.

Itching

There are several treatments available to help with itching. These include antihistamines, antibiotics or a medication called cholestyramine (brand name Questran Lite). If you’re itchy, try to keep your fingernails short. Avoid hot baths and showers, try to wear natural fibres and watch you don’t get too much sun.

Dry eyes and mouth

You can buy artificial tears and saliva over-the-counter from a pharmacy. Chewing gum or sucking a lolly can help.

Diet

It’s important to eat a healthy, balanced diet to improve your overall health and help your liver work properly. Because your body may have trouble absorbing some vitamins, your doctor may recommend supplements such as A, D, E and K, calcium, folic acid or iron.

Fluid retention

You may be given diuretics, medications to help your body control fluid.

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What next?

Read more about living well

Reviewed November 2022