Primary sclerosing cholangitis (PSC)

Usually there aren't any symptoms at first and PSC 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 for many years but when they do arise, symptoms develop gradually over weeks or months.

Symptoms may include:

• fatigue (extreme tiredness)
• feeling generally unwell
• abdominal discomfort in the right upper abdomen
• diarrhoea
• itchy skin
• weight loss
• fever, shaking and chills
• jaundice (yellowing of the skin and the whites of the eyes)

Liver failure may develop. PSC may also be complicated by the development of bile duct cancer.

Your consultant will discuss your symptoms with you and may recommend tests to aid diagnosis. Tests usually include:

• Liver function tests (LFTs), blood tests that measure enzymes and proteins made in the liver. These can indicate liver inflammation and liver function
• An ultrasound scan of the liver.
• A cholangiogram to show a picture of the bile ducts. There are two methods: an endoscopic cholangiogram (ERCP) or a magnetic resonance cholangiogram (MRCP).
• Fibroscan to assess liver fibrosis.
• A liver biopsy where a tiny sample of liver tissue is taken for study. This can show inflammation and the extent of any cirrhosis (scarring), and assess how early or advanced the disease is.

If you've been diagnosed with PSC, your consultant will discuss your treatment options with you and help to determine the best approach.

Currently there is no known cure for PSC, although symptoms such as itching can be treated with ursodeoxycholic acid, cholestyramine, rifampicin or naltrexone. 

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 to dilate the narrowed bile ducts is also an option.

In the late stages of the disease where there is advanced cirrhosis, a liver transplant may be recommended.