Primary biliary cholangitis (PBC)

Some people with PBC will never get any symptoms of the disease. Clear symptoms of PBC are constant fatigue or tiredness (in some this can be severe) and intense itching in any part of the body. Other symptoms include:

• bone and joint ache
• dry eyes and mouth
• indigestion, nausea or poor appetite
• diarrhoea
• dark urine and pale stools
• arthritis (inflammation of the joints)
• mottled palms with red or pink blotches
• pain or discomfort in the upper right side, below the ribs
• dizziness when standing up (postural or orthostatic hypotension)
• jaundice

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

PBC is usually diagnosed through blood tests. However, many people are only diagnosed with the disease after having a routine blood test for another reason. Most people with PBC have a high level of certain liver enzymes in the bloodstream, and an antibody called type 2 antimitochondrial antibody.

Once diagnosed, you'll have an ultrasound scan in order to rule out other problems with your bile ducts, and to assess the condition of your liver.

Transient elastography (FibroScan) to measure scarring levels.

A liver biopsy may also be recommended to assess your liver and help your consultant decide on the best treatment. A biopsy involves removing a small sample of your liver tissue to be studied for signs of disease.

As PBC is a progressive disease, liver damage gets steadily worse over time. Without treatment, liver failure may occur, which can be fatal. There are a number of treatments for managing symptoms of PBC:

• Cholestyramine may be prescribed by your consultant to help ease itching.
• Lozenges, artificial tears and lubricating gels may help dry mouth and eyes.

To slow down disease progression, a medication called ursodeoxycholic acid (URSO, also referred to as UDCA) has been shown to be effective in protecting the liver from the harmful chemicals in the accumulated bile. In people not responding to ursodeoxycholic acid, other medications called fibrates and obeticholic acid are used.

A liver transplant is only recommended if other treatments are no longer helpful.