How is primary biliary cholangitis treated?
If you are diagnosed with PBC you will be under the care of a consultant specialising in liver disorders, and will probably also have access to a specialist nurse for day-to-day advice. There is no specific curative treatment for PBC. The treatments you are offered will depend on your symptoms, on the stage that your disease has reached and on how rapidly things are changing.
Treatment focuses on:
- Treatments to reduce symptoms.
- Treatments to slow the course of the disease.
- Replacement of the liver.
What are the treatment options to stop the itching?
Itch (pruritus) can be a distressing symptom and can be difficult to treat.
- The most commonly used medicine for itch caused by liver disease is colestyramine (trade name Questran®). Colestyramine works by binding to bile in the gut to stop it from making its way back to the liver. When bile gets into the gut, it travels down to the large intestine. Here, some of it is taken (absorbed) back into the bloodstream and returns to the liver to be reused. As colestyramine binds to bile in the large intestine, it stops this reabsorption and so more bile than usual is passed out with the stools (faeces). This helps to reduce the build-up of bile in the liver and bloodstream, which often eases itch. There may be a delay of 1-4 days after starting treatment before itch improves. Other bile-binding medicines are sometimes used.
- Other medicines sometimes used to ease itch if colestyramine is not helpful include antihistamines and an antibiotic called rifampicin. Ursodeoxycholic acid (UDCA) - see below - may also relieve itch.
- Plasmapheresis, which is like a plasma exchange, has been used in some cases of persistent, severe itch. It may need to be repeated but seems to be effective.
- Dry skin can make itch worse, so using liberal amounts of moisturiser is useful if you have dry skin. It can help to keep the moisturiser cool.
- Crotamiton is an anti-itch medication which is sometimes added to moisturiser to help relieve itch. Moisturising creams containing menthol may also be helpful as they give a cooling feeling to the skin.
- Keeping cool will also tend to reduce itch, compared to being warm. Cool showers or cold flannels may be helpful.
- Scratching will generally make it worse, as scratching heats the skin and brings itch-generating substances to the surface. If scratching is irresistible it may help to rub the itchy area with an ice cube, which both satisfies the need to rub it and cools it down at the same time.
- Naloxone (more commonly known as a treatment for opiate overdose) can be helpful.
- The antidepressant sertraline can be helpful both for itch and for low mood associated with tiredness.
What are the treatment options for the tiredness?
- No medicines seem clearly to ease tiredness (fatigue) which is often a main symptom. A medicine called modafinil (usually used in narcolepsy) is sometimes helpful.
- Tiredness is a depressing feeling, particularly when it is unrelenting. If you are affected then finding ways to distract from the physical tiredness, such as by watching films or listening to music, may be helpful. Antidepressants may be helpful, particularly sertraline which can also help with itch.
What treatment slows the progression of the disease?
There is no medicine that stops or reverses the disease. However, some medicines may slow down the progression of the disease in some people:
- Ursodeoxycholic acid (UDCA) is the most common medicine used, with the aim of slowing the progression of the disease. It works by changing the 'makeup' of bile in the liver. This may reduce the harmful effect of bile on the liver cells. UDCA it does not work in everyone. It seems to work best in the early stages of PBC, when it appears to slow disease progression, and may prolong the time for which patients with PBC feel well. It seems to be less effective in the later stages of PBC (in people who have developed scarring of the liver (cirrhosis)). It may help to ease itch and jaundice, as it reduces the levels of some of the increased liver by products in the blood.
- Immunosuppressive medicines are sometimes used, including penicillamine, azathioprine, methotrexate, ciclosporin and steroids. These work by suppressing the immune system. These medicines are sometimes used alongside UDCA, particularly in those patients who don't respond well to it. They also all have a risk of causing significant side-effects, and need to be carefully monitored.
- A new group of medicines called biologics looks promising as treatment for autoimmune diseases like PBC. Research continues in this area.
- Obeticholic acid (Ocaliva®) is a new treatment for PBC. It is used alone or in combination with UDCA. It has not yet been proved to slow disease progression, although it lowers levels of one of the liver enzymes which indicates bile duct blockage. A trial to see if it has long-term benefits is underway but it will take time to prove that it slows progression of a disorder that progresses slowly anyway; results are currently expected in 2023.
What about the diseases associated with primary biliary cholangitis?
Various other autoimmune diseases are more common in people with PBC. You may need treatment for these too.
'Thinning' of the bones (osteoporosis) is more common in women with PBC. The prevention and treatment of osteoporosis are the same as for any other woman. They are discussed in the separate leaflet called Osteoporosis.
What is the treatment for cirrhosis and liver failure?
A liver transplant is an option if the liver becomes badly damaged due to cirrhosis. This is a major operation and is not to be undertaken lightly. However, it can be a life-saving measure and the results are often very good, with around 9 out of every 10 patients recovering extremely well.
Some people with severe itch which has not responded to any other treatment have a liver transplant even if their liver is not too badly damaged. This is because severe itch is an extremely distressing symptom which can severely reduce their quality of life.
Sometimes, PBC can come back and affect the transplanted liver. However, this does not necessarily happen and, if it does, can take up to 15 years before it returns. It is not usually severe, so that another liver transplant does not generally become necessary.
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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