What causes primary sclerosing cholangitis?

The cause of primary sclerosing cholangitis (PSC) remains unknown. Liver damage and cirrhosis is often presumed to be caused by drinking too much alcohol, however, PSC is not related to alcohol in any way. Current evidence suggests that the disease may be triggered by an unknown bacteria or virus in people who are genetically programmed to get the disease. The common viruses known to cause hepatitis have not been associated with it. The frequent occurrence of PSC in association with inflammatory bowel disease suggests that a common cause for both diseases may exist or that the inflamed colon allows toxins or infections to be absorbed into the body and this can cause the bile duct inflammation. The disease affects both genders, although two male patients are affected for every female patient and it can affect all ages.

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What are the symptoms of Primary Sclerosing Cholangitis?

Many people have no symptoms at first and the disease is only discovered because of abnormal results of routine blood tests in patients with ulcerative colitis or Crohn’s disease. In some people PSC does not produce any symptoms. Most people have few or no symptoms for many years.

Common early symptoms of PSC are:

• tiredness
• some abdominal discomfort in the right upper abdomen

Late symptoms of PSC are:

• itching
• jaundice – yellowing of the skin and whites of the eyes
• episodes of fever, shaking and chills can be distressing but are uncommon

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How is Primary Sclerosing Cholangitis diagnosed?

PSC is diagnosed on the combination of symptoms, blood tests and a picture of the bile ducts, called a cholangiogram. A liver biopsy may be needed not only to confirm the diagnosis, but also to assess either how early or advanced the disease is.

If your GP suspects you may have the condition you will be referred to a hospital specialist for tests, expert advice and treatment.

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Can primary sclerosing cholangitis be prevented?

It is not possible to prevent PSC because the cause remains unknown. Liver damage and cirrhosis is often presumed to be caused by drinking too much alcohol, however PSC is not related to alcohol in any way.

Current evidence suggests that the disease may be triggered by an unknown bacteria or virus in people who are genetically programmed to get the disease. The common viruses known to cause hepatitis have not been associated with it.

The frequent occurrence of PSC in association with inflammatory bowel disease suggests that a common cause for both diseases may exist or that the inflamed colon allows toxins or infections to be absorbed into the body and this can cause the bile duct inflammation. The disease affects both genders, although two male patients are affected for every female patient. It can affect all ages, but it is most commonly found in young adults.

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Primary scloerosing cholangitis, infammatory bowel disease, and liver cancer

PSC may be occasionally complicated by the development of bile duct cancer. PSC is closely associated with inflammatory bowel disease, usually ulcerative colitis, but also Crohn’s disease. The course of the ulcerative colitis is often very mild, with few flare-ups. However bowel cancer does appear to develop more frequently in patients with colitis who also have PSC and regular yearly examination of the colon by colonoscopy (a tiny video camera) is recommended.

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Treatment for Primary Sclerosing Cholangitis

At present there is no known cure or specific treatment for PSC. Clinical trials are happening to try to find treatments for the future.

Symptoms such as itching can be treated with agents such as cholestyramine, rifampicin or naltrexone.

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Living with primary sclerosing cholangitis

When someone has a long-term liver problem such as PSC, the liver’s ability to carry out its many functions may not be as good as in a healthy person. In particular, the liver’s ability to break down alcohol and medicines may be impaired, however this is often not a problem. If in doubt, check with your doctor.

Diet

People with PSC may not have any symptoms associated with fat malabsorption, while others develop symptoms of fat intolerance such as nausea, bloating and abdominal discomfort if they eat even moderate amounts of dietary fat. As the disease advances, the amount of fat excreted in the stools increases (steatorrhoea) and they become bulky, pale and have a tendency to float in the toilet pan.

People with PSC vary in the amount of fat they can tolerate. Most find they are able to work out their tolerance level through ‘trial and error’ by reducing their intake of higher fat foods. Stools become less frequent, darker and easier to flush away. If, however, steatorrhoea is still troublesome then specialist dietary advice will be needed in order to reduce dietary fat intake while keeping energy and protein levels relatively high.

Fats contain the fat-soluble vitamins A, D, K and E, and these may need to be supplemented. Oral supplements can be given but as the disease progresses it may be necessary to provide the supplements monthly by intramuscular injection.

As people with PBC and PSC are prone to bone disease, calcium supplements will usually be given alongside vitamin D.

People with PBC and PSC may develop cirrhosis and need to follow special diet advice. Read more about diet and cirrhosis here.

Tiredness (fatigue)

Tiredness is the commonest symptom of PSC. Some people may need to consider making changes to their lifestyle and pacing their daily activities helps to preserve stamina and energy. Gentle exercise such as walking and swimming can be beneficial.

Find out more about  coping with fatigue

Talk to your dentist and doctor

Do tell your dentist that you have PSC as there may be an increased risk of bleeding.

It is important that you talk to your doctor before taking any medicine (not prescribed by him or her), including paracetamol, herbal remedies and Chinese medicine.

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The UK-PSC study

UK-PSC is a collaborative venture between NHS Trusts across the UK, researchers and patient groups aimed at improving our understanding of PSC and the way it impacts on the lives of patients. People of all ages (including children) with PSC are encouraged to join. Visit www.uk-psc.com.

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