Primary Sclerosing Cholangitis (PSC)

PSC Facts

What is PSC?
What causes PSC?

Primary Sclerosing Cholangitis is an uncommon chronic liver disease in which the bile ducts inside and outside the liver progressively decrease in size due to inflammation and scarring (fibrosis). The disease may occur alone, but frequently is associated with inflammatory diseases of the colon, especially chronic ulcerative colitis.

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.

PSC: symptoms & diagnosis

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.

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.

Liver failure may ultimately develop

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.

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.

Cholestyramine

Cholestyramine (trade name Questran® or Questran Light®) may be prescribed to help relieve itching. It works better when taken before and after meals, especially breakfast, but it may take some days before the treatment is effective. Some people taking Questran® experience side effects such as altered bowel habits or bloating. If this is a problem, tell your doctor as there are other treatments such as albumin dialysis that may suit you better.

Many people suffer with a dry mouth and dry eyes, but this can be helped by taking lozenges and artificial tears prescribed by your doctor.

Liver transplant

For a few people who eventually go on to get advanced cirrhosis, a liver transplant may be recommended when their quality of life has deteriorated and medical treatment can no longer control their symptoms. Because PSC usually develops slowly, transplantation can generally be carefully planned well ahead.

Endoscopic treatment

People with advanced PSC are often deficient in vitamins A, D and K and replacement fat-soluble vitamins are given. Endoscopic treatment of the bile ducts is aimed at reducing or halting the progression of the disease, usually by stretching or dilating the narrowed bile ducts. In later stages of the disease liver transplantation is often an option. Survival after this operation is good, although in some patients PSC can recur in the new liver.

Further Information

Can Primary Sclerosing Cholangitis (PSC) 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.

Looking after yourself when you have Primary Sclerosing Cholangitis (PSC)

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.

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.

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

Please visit the support section of our website for information on Support groups in your area.

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