Primary sclerosing cholangitis (PSC)

Autoimmune Liver Diseases (AILD)

Description

Primary sclerosing cholangitis (PSC) is a rare, immune-mediated liver disease that leads to scarring in the bile ducts. This means that bile, which normally flows through the bile ducts into the intestines, can’t drain properly and it builds up in the liver. This can lead to infection and liver damage in some people.

Diagnosis

PSC can be difficult to diagnose.
Routine Blood Tests
The diagnosis often comes unexpectedly after routine blood tests for IBD suggest bile duct injury. In other cases, people suffer with fatigue or feel generally unwell, or may show signs of liver disease such as itch or jaundice (yellowing of the eyes/skin), which triggers investigations to identify the cause.
MRI Scan
If your doctor suspects PSC, you will be asked to have a type of MRI scan called an MRCP scan. An MRCP looks closely at your bile ducts.
Other Tests
Where the MRCP shows a typical appearance for PSC, invasive tests are rarely needed. However, occasionally a biopsy is taken when other conditions including associated autoimmune hepatitis are suspected. Only in rare cases is an endoscopic cholangiography (ERCP) required to establish the diagnosis.

Management

What can my doctor do?

While there is currently no curative treatment for PSC, your doctor can help manage your symptoms using current clinical practice guidelines. This includes medication to treat itch, antibiotics to treat infections and vitamin supplements.Some people with PSC take ursodeoxycholic acid (UDCA) at low dose (15 to 20 mg per kg body weight per day). It is not clear whether UDCA delays progression of PSC, although it does improve liver blood tests and may help improve the flow of bile.People with PSC face an increased risk of some forms of cancer. Your doctor should keep a close eye on you and monitor you regularly.
New therapies for PSC are currently being tested. Your doctor can talk to you about opportunities to take part in research trials to access potential new drugs.

What tests or procedures will I have?

  • An ultrasound scan every 6-12months to look at the shape of your liver, bile ducts and gallbladder.
  • A colonoscopy at diagnosis to check for the presence of IBD, even if you have no symptoms. If you do have IBD, expect to have yearly colonoscopies.
  • A bone density scan to check your bones, as PSC is associated with an increased risk of osteoporosis.
  • Endoscopic treatment (ERCP) can sometimes be used to physically widen narrowed bile ducts to help the bile flow.
  • Regular liver blood tests to monitor your liver enzymes.
  • A FibroScan® every year (if available) to look at scarring in the liver.
  • An MRI scan of your bile ducts (MRCP) if you have new or changing symptoms or changes are seen in other tests.