Primary Sclerosing Cholangitis

What is primary sclerosing cholangitis?

Primary sclerosing cholangitis, also known as PSC, is a rare condition which affects the liver. In people with PSC the bile ducts within and on the exterior of the liver become progressively smaller, causing bile to collect in the liver. Bile is a yellowy-green fluid made by the liver, stored in the gallbladder and transported via the bile ducts. It is responsible for breaking up fat from foods during the digestion process, by breaking the fat into small, manageable droplets, which can then be absorbed by the body. Bile also enables the body to absorb important vitamins, including vitamins A, D, K and E.

If bile starts to collect in the liver as a consequence of the bile ducts becoming blocked, this can cause liver damage, inflammation and scarring. In most cases the liver cells are able to re-grow without any problems; however, in people with PSC this is not possible and the healing process cannot occur properly

The condition can become apparent on its own, but it is frequently linked to other conditions that affect the colon, including ulcerative colitis. PSC can affect people of all ages but it tends to be more widespread in adults aged between 30 and 60. The condition is slightly more widespread in males than females.

What causes primary sclerosing cholangitis?

The chief cause of the condition is not fully understood. Researchers are currently investigating possible causes, which may include the role of bacteria and viruses and problems affecting the immune system.

Symptoms of primary sclerosing cholangitis

It can take a long time for symptoms to develop. Accordingly many cases are not diagnosed until they have blood tests for other related conditions, such as Crohn’s disease or ulcerative colitis. Symptoms in the early stages include fatigue, feeling lethargic and abdominal pain in the upper right hand side of the abdomen.

At a more advanced stage symptoms may include:

• Jaundice (when the skin and whites of the eyes become yellow).
• Itching.
• Fever (a high temperature).
• Chills and the shakes (this is not common).

Complications of primary sclerosing cholangitis

Primary sclerosing cholangitis can lead to liver failure and is also linked to an increased risk of bowel cancer. It is usually associated with other inflammatory conditions affecting the pelvis, including ulcerative colitis and Crohn’s disease. Doctors advise patients with ulcerative colitis and PSC to have regular endoscope tests to ensure that any signs of cancer are detected as early as possible.

How is primary sclerosing cholangitis diagnosed?

After talking to you about your symptoms and examining you, your GP will refer you to a specialist gastroenterologist if they suspect you have PSC. A combination of tests is usually carried out to diagnose primary sclerosing cholangitis, which usually include:

• Liver function tests: these blood tests indicate how well the liver is working.
• Liver biopsy: this involves taking a small sample of tissue, which is then sent to the laboratory for analysis under a microscope.
• Ultrasound scan: the bile ducts can be scanned to allow doctors to see the ducts and rule out other conditions.
• Cholangiography: this test shows images of the bile ducts. There are two ways of carrying out the test, including using an endoscope and injecting coloured dye into the bile ducts to make X-ray images clear, and using an MRCP (magnetic resonance cholangiogram), which is a newer method and requires the patient to lie in a scanning machine.

Treatment for primary sclerosis cholangitis

There is currently no cure for primary sclerosis cholangitis; however, there are treatments available to reduce symptoms and slow down damage to the liver. Trials have suggested that natural bile acid may be effective in preventing or slowing damage to the liver by speeding up the flow of bile. People who have PSC often suffer from vitamin deficiency as a consequence of being unable to absorb key vitamins from their diet, so supplements of vitamins K, A and D are usually prescribed. A procedure to widen the bile ducts, using an endoscope, can also help to slow down the progression of the condition.

In extreme cases, when scarring of the liver (known as cirrhosis) is severe, a liver transplant may be the only option. A liver transplant involves replacing a badly damaged liver with a healthy liver from another person. PSC tends to develop slowly and transplants can be planned well in advance; however, operations can only be carried out if a healthy liver becomes available.

Self-help

Anyone with a long-term liver problem will be advised to take steps to ease symptoms and prevent further damage to their liver. The liver may not be able to function as effectively and this means that you should avoid putting unnecessary strain on it. You will probably be advised to cut right down or avoid drinking alcohol altogether if you are diagnosed with a liver condition. Some forms of medication may cause problems and it is important to check with your doctor beforehand. Most people are able to eat a normal diet, but if you have any queries or need help with your diet, contact your doctor.