Primary Sclerosing Cholangitis
Primary sclerosing cholangitis is an uncommon condition that affects the bile ducts and liver. Inflammation and scarring of the bile ducts can lead to liver damage and cirrhosis - a condition where normal liver tissue is replaced by scar tissue (fibrosis).
Different treatments are available to control the symptoms of primary sclerosing cholangitis that may develop and also to manage any complications which can occur. The outlook for people who are suffering with primary sclerosing cholangitis can be very variable.
Primary sclerosing cholangitis is an uncommon condition that mainly affects the bile ducts and liver. It is called:
- Primary - as the cause is not known.
- Sclerosing - as it causes scarring and thickening (sclerosis) of the bile ducts.
- Cholangitis - it means inflammation of the bile ducts.
In this condition, the bile ducts of 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.
Eventually, the scar tissue can spread throughout the liver, causing cirrhosis and liver failure. Cirrhosis is a serious condition in which the normal liver tissue is replaced by scar tissue (fibrosis).
This tends to progress slowly and often does not cause any symptoms in its early stages. So, as the function of the liver gradually becomes worse, serious problems can develop too.
How is Primary Sclerosing Cholangitis diagnosed?
Many people with primary sclerosing cholangitis have no symptoms for quite some time in the early stages of the disease. So, the diagnosis is often done when you have tests for an unrelated condition or routine tests if you have inflammatory bowel disease.
Generally tests include:
- Blood tests called liver function tests - These measures the activity of chemicals (enzymes) and other substances made in the liver.
- Different blood tests can be performed to rule out (exclude) other causes of liver conditions such as viral hepatitis.
- An ultrasound scan of the liver may be performed.
- 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. The liver biopsy can also assess how early or advanced the disease is.
What are the symptoms of Primary Sclerosing Cholangitis
In many cases the symptoms develop gradually over weeks or months. At the beginning of the disease, many people have no symptoms at all.
Jaundice can be developed when the condition gets worse. If you have jaundice, you 'go yellow'. You tend to notice it first when the whites of the eyes become yellow. This is due to a build-up of the chemical bilirubin, which is produced in the liver and, in some liver conditions, spills into the blood.
What is the treatment of Primary Sclerosing Cholangitis?
At present, there is no specific treatment which either treats or slows the progression of primary sclerosing cholangitis. Treatments focuses to improve symptoms and also to manage any complications which may arise.
Treatment of symptoms
Itching of the skin can be difficult to treat but is generally eased with a medicine called colestyramine. Other medicines can be tried if this is not effective.
Still there is no effective medical treatment available apart from using medicines to ease symptoms. Vitamin supplements can be recommended.
Treatment of complications
Antibiotics will be given if you have an episode of infective cholangitis. If your cholangiogram shows that you have an obstruction to the bile duct outside your liver, it may be possible for a doctor to place a small drainage tube (a stent) or use a very small balloon to open up the blockage.
If your liver is affected badly then you may be considered appropriate for a liver transplant. Outlook after a liver transplant is good. Though, primary sclerosing cholangitis recurs in around a fifth of cases after a liver transplant.