By Dr Saravana K.
Consultant Physician, Gastroenterologist & Hepatologist
PSC is an uncommon condition affecting the bile ducts and liver.
Primary – because the cause is not known.
Sclerosing – because it causes scarring and thickening (sclerosis) of the bile ducts.
Cholangitis – which means inflammation of the bile ducts.
In this condition, the bile ducts both inside and outside the liver become inflamed and scarred. The scarring causes narrowing of these bile ducts which results in bile building up in the liver. The bile can then damage the liver cells. Bile is a yellow-green liquid that helps you to digest food, particularly fatty foods. It also helps the body to absorb certain vitamins (A, D, E and K) from the food that you eat.
Eventually, the scar tissue can spread throughout the liver, causing cirrhosis and liver failure. Cirrhosis is a serious condition where normal liver tissue is replaced by scar tissue (fibrosis). It tends to progress slowly and often does not cause symptoms in its early stages. However, as the function of the liver gradually becomes worse, serious problems can develop.
The cause is not clear. It can sometimes run in families. Around four out of five people with PSC also have inflammatory bowel disease.
PSC is rare. It can occur at any age but is more common in people aged around 40 years. It is twice as common in men as in women.
In many cases the symptoms develop gradually over weeks or months. The most common early symptoms include feeling more tired than usual, feeling generally unwell, itchy skin, weight loss and having some discomfort in the right upper abdomen. Jaundice can develop when the condition worsens.
Various complications can occur in some people with PSC. These include:
Deficiencies of some vitamins, usually vitamins A, D, E and K.
Infection of the blocked bile ducts. This is called infective cholangitis.
Bile duct cancer. This eventually affects around one in 10 people who have PSC.
Increased risk of colorectal cancer.
How is primary sclerosing cholangitis (PSC) diagnosed?
Tests usually include:
Blood tests called liver function tests.
Other blood tests may be performed to rule out (exclude) other causes of liver conditions such as viral hepatitis.
A cholangiogram is a test which produces a picture of the bile ducts. This is often done using an MRI scan.
Taking a small sample (biopsy) of the liver. This may be carried out to look at the sample under the microscope. It can show inflammation and the extent of any cirrhosis (where normal liver tissue is replaced by scar tissue [fibrosis] in the liver).
At present there is no specific treatment which either cures or slows the progression of PSC. Treatments aim to improve symptoms and also to manage any complications which may arise.
Itching of the skin can be difficult to treat but is usually eased with a medicine called colestyramine. Vitamin supplements may be advised.
Antibiotics will be given if you have an episode of infective cholangitis.
If your liver is affected badly then you may be considered for a liver transplant. Outlook following a liver transplant is good. However, PSC recurs in around a fifth of cases following a liver transplant.