Primary sclerosing cholangitis (PSC)

What is PSC?

Primary sclerosing cholangitis, or 'PSC', is a chronic liver disease in which the bile ducts, which are the tubes that carry bile from the liver to the small intestines, become narrowed from inflammation/swelling and scarring called fibrosis. Over time, the scar tissue builds up and can block parts of the bile ducts completely. The blockages can occur in any part of the bile duct system/tree: inside the liver itself (intrahepatic) or outside the liver (extrahepatic). PSC can rarely lead to liver cancers and so requires monitoring by liver specialists.

What causes PSC?

PSC is an auto inflammatory disease, in which the immune system “misfires” and bile ducts become inflamed. No one knows why this happens. Some people may have a higher genetic risk, and those with other autoimmune conditions may also be more likely to be affected by PSC. PSC more often occurs in men than women. The age at which it is diagnosed in most patients is between 25 and 60 years, though may occur at any age. There is a link with inflammatory bowel disease.

What are the symptoms of PSC?

PSC often causes no symptoms and is identified because of abnormal liver blood tests. The symptoms that PSC can cause are tiredness, itching, yellow skin (jaundice) and fevers.

What treatment will I receive for PSC?

PSC is a complex condition that affects everyone differently. There is no proven medication for it. The main treatment for PSC is to monitor patients for problems caused by it. Therefore, you will have an outpatient appointment every six to 12 months.

You will have an ultrasound scan of the liver every 6 to 12 months. Regular ultrasound scans are important to monitor changes in the condition and, if the disease is at a more advanced stage, provide surveillance for liver and gallbladder cancer to help detect these at a treatable stage. People with PSC and inflammatory bowel disease (IBD) should have a colonoscopy every year. Annual colonoscopies are important because people with PSC and IBD have increased risk of colon cancer; regular colonoscopies can help to detect colon cancer at an early and treatable stage.

Some patients with PSC may be prescribed a medication called ursodeoxycholic acid (UDCA), but the effectiveness of this medication is unproven. It may be taken in divided doses or all at once. Some patients with PSC may eventually need a liver transplant.

Whilst we will do our best to keep the monitoring of your PSC up to date, it is useful for you to be familiar with the programme we recommend and encourage you to contact us if you feel that a test request may have been overlooked.

Symptoms can be variable and should not be ignored

Please make contact if you develop any of the following symptoms/problems, as they may indicate a deterioration in your condition: itching, pain, jaundice, dark urine, pale stools, high fever.

Bacterial cholangitis

Bacterial cholangitis is a condition where bile ducts become infected, usually because of a hold up in bile flow. Bacterial cholangitis is unpredictable, but only happens at more advanced stages of PSC. Symptoms include fever, shivers, chills, abdominal pain, nausea, vomiting and jaundice.

If you think you have bacterial cholangitis, urgent medical care is required so you will need to be assessed by A&E, your GP or hepatology doctor. You will usually be administered an antibiotic to treat the infection. Please inform your liver team if you have needed antibiotics from elsewhere, as further investigations are often recommended.

Long term prognosis / outcome

Internet searches for PSC often give worrying survival figures. Fortunately, new research provides much more reassuring estimates. Indeed, many patients who only have the disease in the small bile ducts never run into problems. Specific outcome for you can be discussed with your doctor.

What research is happening in PSC

There may be opportunities to be involved in clinical trials for new treatments of PSC. If you are interested in taking part in this research or would like to discuss it, please contact us on 01223 256225 or e-mail Hepatology Research.

Contacts

If you require any further information about this leaflet or advice about your condition please contact the Addenbrooke’s team on 01223 216109 or 01223 596094

Where can I receive further information and support?

• British Liver Trust (opens in a new tab): Helpline 0800 652 7330 – 10:00 (10am) to 15:00 (3pm) Monday to Friday
• UK Primary sclerosing cholangitis (opens in a new tab)
• PSCS support group (opens in a new tab)