Primary biliary cirrhosis

Overview

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.Sometimes, 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. Occasionally, if the liver is severely damaged, a liver transplant may be needed.

Complications of PBC

If PBC is not treated or reaches an advanced stage, there may be other problems including:

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

Symptoms

Most people are only diagnosed with PBC after having a routine blood test for another reason. Some people have symptoms early on and some may develop them later.

People who have symptoms may experience:

bone or joint aches
fatigue – this is a common symptom (but not always caused by PBC) and can have a significant impact on your daily activities
itchy skin – this can be widespread or 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 side of the tummy
dizziness when standing up (postural or orthostatic hypotension)

Symptoms can range from mild to severe, and this isn’t always related to the degree of liver damage you have.

Some people with PBC have severe symptoms but their liver isn't severely damaged, while others may have significant liver damage but no symptoms or only mild ones.

Some people with PBC may also have symptoms of another condition, such as an underactive thyroid (hypothyroidism).

Advanced PBC

Medicine can usually help to delay liver damage caused by PBC.

But as the liver slowly becomes more scarred and damaged (eventually ending up with cirrhosis), you may have additional symptoms.

These can include:

yellowing of the skin and whites of the eyes (jaundice)
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

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) like aspirin and ibuprofen.

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

Ursodeoxycholic acid

Ursodeoxycholic acid (UDCA) is the main treatment for 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. But it does not improve symptoms such as itchy skin or fatigue.

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

You may need regular blood tests to monitor your liver function, and you'll be assessed after a year to see if you've responded to UDCA. If not, you may need additional treatment.

Side effects

Weight gain is a possible side effect of UDCA. People gain an average of 2.3kg (5lbs) during the first year of taking the medicine, although there's not usually any more weight gain after this.

Other side effects can include diarrhoea, feeling and being sick, 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 is not working well enough
on its own – for people who cannot take UDCA

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

Side effects

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

If you have very advanced liver disease, you 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're 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're taking colestyramine long-term, it may affect your body's ability to absorb vitamins A, D and K from food. Taking additional vitamin supplements may be beneficial.

A GP can advise you on whether you would benefit from taking vitamin supplements.

Alternative treatments for itching

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

Alternative medicines are available, such as an antibiotic called rifampicin and a medicine 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

No medicines are currently 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

A liver transplant may be recommended if it's thought 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. This is because:

the national average waiting time for a liver transplant is 65 days for adults, 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. Your 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.

Most people live more than 10 years after having a liver transplant and many live for up to 20 years or more.

But having a liver transplant does not always completely cure PBC. Around 1 in 5 people who have a liver transplant for PBC develop the condition in their new liver. This is known as recurrent PBC.