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 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 side of the tummy
• dizziness when standing up (postural or orthostatic hypotension)

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

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

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.

Advanced PBC

Medication can usually help to delay liver damage in people with 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

Read more about the symptoms of cirrhosis.

The aim of treatment for PBC is to slow down the liver damage 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 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'll need to take it for the rest of your life.

Regular blood tests may be carried out to monitor your liver function, and you'll be assessed after a year to see if you have responded well to it. 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's a good idea to take the powder with fruit juice because it has an unpleasant taste.

You may need to take colestyramine for up to a few weeks 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 1 hour 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 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 doesn't 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

A liver transplant may be recommended if it's thought the liver damage may eventually put your life 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 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.

It's now common for people to still be healthy and well more than 20 years after a liver transplant.

There's 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.