Primary biliary cholangitis (PBC) is a condition that slowly damages the bile ducts in the liver, leading to liver damage and, sometimes, liver scarring (cirrhosis).
What is primary biliary cholangitis?
Primary biliary cholangitis (PBC) is an auto-immune condition in which the immune system gradually destroys the tiny tubes (bile ducts) which take bile from the liver to the gut (intestine). The trapped bile then builds up in the liver, where it causes inflammation and damage to liver cells. This can eventually (usually after many years) lead to scarring of the liver (cirrhosis).
Primary biliary cholangitis is called:
- Primary - because it is not secondary to any known cause such as alcohol.
- Biliary - because it affects the bile ducts.
- Cholangitis - because there is inflammation of the bile ducts.
Read more about the cause of primary biliary cirrhosis.
Who gets primary biliary cholangitis?
PBC is an uncommon condition. It affects about 1 in 5,000 people in the UK. 9 out of 10 cases occur in women, who are mainly aged between 30 and 65 years (most commonly between 40 and 60 years). It is most common in the Northern Hemisphere and least common in Australia.
What are the symptoms?
There may be no symptoms of PBC at first, and for a long time. Common early symptoms are itch and tiredness. Other symptoms develop as the disease progresses, and may include symptoms of liver inflammation, yellowing of your skin or the whites of your eyes (jaundice) and, at worst, liver failure.
Learn more about the symptoms of PBC.
How is it diagnosed?
PBC is normally diagnosed using blood tests and scans. These are usually done if you are tired or itchy, but the condition may be picked up incidentally when you have a blood test for some other reason.
Find out more about the diagnosis of PBC.
What is the treatment?
There is no specific cure for PBC. Treatments aim to ease symptoms and to slow the progression of the disease. In cases where the liver progresses to severe scarring (cirrhosis) and liver failure, liver transplant can be life-saving.
Learn more about the treatment of PBC.
What is the outlook?
PBC is a progressive disease. Most commonly it progresses very slowly over a number of years. Most people have very few problems for twenty years or more.
The outlook is less good for those who already have jaundice at the time of diagnosis, as their disease has already progressed. They are more likely to need liver transplant within five years.
What can I do to help myself?
PBC is a slowly progressing disease and if you are diagnosed early you are likely to stay healthy for many years. Your lifestyle choices around fitness and diet may help keep you healthy for longer.
Read more about staying healthy when you have PBC.
What are the possible complications?
The most serious complication of PBC is liver failure, which does not occur in everyone. It is impossible to predict, early in the disease, which patients are likely to experience this.
Discover more about the complications of PBC.
Is primary biliary cholangitis the same as primary sclerosing cholangitis?
Primary biliary cholangitis (PBC) is not to be confused with primary sclerosing cholangitis (PSC). Both conditions cause problems with the bile ducts but PSC mainly affects men and affects a younger age group than PBC. This leaflet is about primary biliary cholangitis. For information on PSC, see separate leaflet called Primary Sclerosing Cholangitis.
Further reading and references
EASL Clinical Practice Guidelines: The diagnosis and management of patients with primary biliary cholangitis; European Association for the Study of the Liver (2017)
Management of cholestatic liver diseases; European Association for the Study of the Liver (June 2009)
I J Beckingham and S D Ryder; ABC of diseases of liver, pancreas, and biliary system: Investigation of liver and biliary disease. BMJ 2001322:33-36.
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