Overview

Primary biliary cirrhosis
Primary biliary cirrhosis

Primary biliary cholangitis (often referred to as primary biliary cirrhosis) is a type of liver disease that can get gradually worse over time. Without treatment, it may eventually lead to liver failure.

Symptoms of PBC

PBC doesn't always cause symptoms, but some people may experience:

  • bone and joint aches
  • fatigue
  • itchy skin
  • dry eyes and mouth
  • pain or discomfort in the upper right side of their tummy

How PBC is diagnosed

Many people are only diagnosed with PBC after having a routine blood test for another reason. PBC can usually be diagnosed just using blood tests.

Once PBC is diagnosed, you'll also need an ultrasound scan to help rule out other problems with your bile ducts and assess your liver.

A liver biopsy is occasionally recommended to assess your liver and help doctors decide on the best treatment.

This involves safely removing a small sample of liver tissue so it can be studied under a microscope.

Causes of PBC

Bile is a liquid produced inside the liver that's used to help digest fats and remove waste products from the body. It passes out of the liver through small tubes called bile ducts.

In PBC, the immune system (the body's natural defence against infection and illness) mistakenly attacks the bile ducts.

It's not clear why this happens, but it's thought to be caused by a combination of subtle differences in how the immune system works.

The bile ducts become damaged and injured, causing bile to build up in the liver. This further damages the liver and may lead to scarring (cirrhosis).

How PBC is treated

PBC is a progressive condition, which means the damage to the liver can steadily get worse over time.

The rate at which PBC progresses varies between individuals. In some cases, it can take decades.

Without treatment, the liver can become so badly damaged that it no longer works properly. This is known as liver failure and can be fatal.

Liver failure can be prevented in the majority of people being treated for PBC with current treatments such as ursodeoxycholic acid and obeticholic acid.

Other medications can help relieve the itchiness associated with PBC. A liver transplant may be required in severe cases, but this is needed less commonly.

Complications of PBC

If PBC isn't treated or reaches an advanced stage, it can be associated with other important problems.

These may include:

  • osteoporosis – a condition in which the bones become weak and brittle
  • portal hypertension – increased blood pressure inside the blood vessels in your abdomen
  • ascites – a build-up of fluid in your abdomen (stomach) and around your intestines
  • vitamin deficiencies – including vitamins A, D, E and K
  • a slightly increased risk of developing liver cancer
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Symptoms

Primary biliary cirrhosis (PBC) often doesn't cause any symptoms in the early stages and many people are only diagnosed with the condition during tests carried out for another reason.

People who do have symptoms may experience:

  • fatigue (extreme tiredness) – this is the most common symptom, and can have a significant impact on your daily activities
  • itchy skin– this can be widespread or it can only affect a single area; it may be worse at night, when in contact with fabrics, when warm, or during pregnancy
  • dry eyes and mouth
  • problems sleeping at night and feeling very sleepy during the day
  • pain or discomfort in the upper right corner of the tummy
  • dizziness when standing up (postural or orthostatic hypotension)

Some people may also have symptoms of conditions that often occur alongside PBC, such as an underactive thyroid (hypothyroidism).

Read about the causes of PBC for more information about related conditions.

For unknown reasons, there is often little association between the severity of symptoms and the degree of underlying liver damage. Some people with PBC have severe symptoms even though tests show that their liver is not severely damaged, while tests may reveal that others with no or mild symptoms may have significant liver damage.

Advanced PBC

Medication can usually help to delay liver damage in people with PBC, but if the liver does become extensively damaged (cirrhosis), you may have additional symptoms.

These can include:

  • jaundice (yellowing of the skin and whites of the eyes)
  • a build-up of fluid in the legs, ankles and feet (oedema)
  • build-up of fluid in your tummy that can make you look heavily pregnant (ascites)
  • the formation of small fatty deposits on the skin, usually around your eyes (xanthelasmata)
  • dark urine and pale stools
  • a tendency to bleed and bruise more easily
  • problems with memory and concentration

Read more about the symptoms of cirrhosis.

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Treatment

The aim of treatment for primary biliary cirrhosis (PBC) is to prevent liver damage as much as possible and reduce your symptoms.

Self-help

All types of liver disease, including PBC, can be helped by following some general health advice.

You should:

  • stop smoking
  • maintain a healthy weight
  • not regularly drink more than 14 units of alcohol a week - read more about alcohol misuse

Following this advice could:

  • help prevent PBC getting worse
  • mean all the treatments for PBC are available to you if you need them – for example, a liver transplant

Avoid certain medications

The damage to your liver can affect its ability to process certain medications.

This means you may need to avoid certain medicines – for example, non-steroidal anti-inflammatory drugs (NSAIDs) such as aspirin and ibuprofen.

Ask your doctor about any medications you should avoid, and inform any health professional treating you that you have PBC.

Ursodeoxycholic acid

Ursodeoxycholic acid (UDCA) is the main treatment for people with PBC. 

It can help prevent or delay liver damage in most people – particularly if you start taking it in the early stages of the condition – although it doesn't improve symptoms such as itchy skin or fatigue.

Once you start taking UDCA, it's likely you will need to take it for the rest of your life.

Regular blood tests may be carried out to monitor your liver function and you will be assessed after a year to see if you have responded sufficiently. If not, you may need additional treatment.

Side effects

One possible side effect of UDCA is weight gain. People gain an average of 2.3kg (5lbs) during the first year of taking the medication, although most people don't gain any more weight after this.

Other side effects can include diarrhoea, nausea, vomiting and thinning hair.

Obeticholic acid

Obeticholic acid (OCA) is a new treatment that works by improving bile flow and reducing inflammation.

It may be offered as an option for treating PBC, either:

  • in combination with UDCA – if UDCA isn't working well enough
  • on its own – for people who can't take UDCA

People who may need OCA will first need assessment by a specialist team with experience in treating PBC.

Side effects

One possible side effect of OCA is itching. If itching is a problem, your dose may be lowered or you may be offered medication to treat the itching.

People with very advanced liver disease may be prescribed a lower dose of OCA, or it may not be recommended.

Treating itchiness

Colestyramine (previously called cholestyramine) is a medicine widely used to treat the itchiness associated with PBC.

It usually comes in sachets of a powder that can be dissolved in water or fruit juice. It is a good idea to take the powder with fruit juice, because it has an unpleasant taste.

You may need to take colestyramine for several days before your symptoms begin to improve.

If you are taking UDCA as well as colestyramine, you should avoid taking them at the same time because colestyramine will affect how your body absorbs UDCA. 

You should take UDCA at least 1hour before colestyramine, or 4 to 6 hours afterwards.

This also applies to any other medicines you're taking. Your GP can give you further advice about this.

Constipation is a common side effect of colestyramine, although this usually improves once your body gets used to the medicine. Other side effects can include bloating and diarrhoea.

If you are taking colestyramine on a long-term basis, the medicine may affect your body's ability to absorb vitamins A, D and K from food.

In such cases, taking additional vitamin supplements may be beneficial. Your GP can advise you on whether you would benefit from taking vitamin supplements.

Alternative treatments for itching

See your GP if your itchiness does not improve after taking colestyramine.

Alternative medicines are available, such as an antibiotic called rifampicin and a medication called naltrexone.

These are usually only prescribed by the specialist that looks after your PBC - for example, a gastroenterologist or hepatologist (liver specialist).

Regularly using moisturisers to stop your skin becoming dry may also help reduce itchiness.

Managing fatigue

At the moment, no medications are available to specifically treat  fatigue associated with PBC.

Your GP may want to rule out and treat any other possible causes for your tiredness, such as anaemia (a lack of red blood cells), sleep apnoea, or depression.

You should try to exercise whenever possible, but you may need to pace yourself and limit your daily activities to a manageable level.

Adjusting your daily routine around fatigue (which is often worse later in the day) can make a big difference to your quality of life.

If you have difficulty sleeping, sleep hygiene measures may help. These include avoiding caffeine, nicotine and alcohol late at night, and maintaining a comfortable sleeping environment.

Treating dry mouth and eyes

If you have a dry mouth and eyes, your GP may recommend eye drops containing "artificial tears" or saliva substitute products.

You should maintain good oral hygiene and visit your dentist regularly because having a dry mouth can increase your risk of tooth decay.

Liver transplant

liver transplant may be recommended if it's thought that the liver damage may eventually put your life is at risk.

Planning for a liver transplant often begins before significant damage to the liver has taken place because:

  • the average waiting time for a liver transplant is 145 days, so it's important your specialist doctor considers assessment and listing for transplantation as soon as possible
  • the better your general state of health, the greater the chance of a successful transplant, so a transplant should ideally be carried out while you're still relatively healthy

Having a liver transplant will cure the itchiness and other symptoms, but you may still have fatigue.

Like all organ transplants, liver transplants carry a risk of complications. The immune system may reject a donated liver, which could be life threatening.

Medicines that suppress the immune system are very effective at preventing this, but they need to be taken for life to reduce this risk.

 Studies have found that more than 9 out of 10 people who have a liver transplant for PBC are still alive after a year and more than 8 out of 10 will live at least another 5 years.

There is a risk of PBC developing in your new liver, but this isn't usually a major concern because it often takes a long time to develop.

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The information on this page has been adapted by NHS Wales from original content supplied by NHS UK NHS website nhs.uk
Last Updated: 21/10/2020 14:10:54