Primary Sclerosing Cholangitis

What is Primary Sclerosing Cholangitis?

Primary Sclerosing Cholangitis or PSC is a bile duct disease. Bile ducts connect the liver with the small intestine and the gallbladder. These tube-like structures transport bile, a liquid made in the liver to help the body digest certain vitamins and fats. The body can only handle so much bile before it begins to get sick. This is why healthy bile ducts are so important.

It is unknown what causes PSC but three-quarters of PSC patients also suffer from an inflammatory bowel disease such as ulcerative colitis. Why these two conditions seem to pop up together is unknown. People of all ages, including children, can get PSC but adults over 40 are the most common patients. Men get PSC more than women for an unknown reason.

What are the Symptoms of Primary Sclerosing Cholangitis?

Symptoms come on so gradually that patients may have PSC and never know it for years. Symptoms then become so annoying that medical help is finally sought out. The most common symptoms are itchy skin, yellowing of the skin, yellowing of the whites of the eyes and feeling tired all of the time.

In later stages of the disease, patients also have to deal with abdominal pain, fever and chills no matter what the temperature is.

Ignore the symptoms can have fatal consequences. If left untreated, the liver will fail or develop cancer.

Primary Sclerosing Cholangitis Causes

Primary sclerosing cholangitis (PSC) is caused by inflammation and fibrosis of the bile ducts which leads to bile ducts becoming blocked. This is what leads to an accumulation of bile in the liver and subsequent liver damage and cirrhosis. However, it’s not clear what causes the initial inflammation and blockage of the bile ducts.

Some experts believe that bacterial or viral infections could cause PSC. As the body tries to fight off an infection, tissues in the affected area can become inflamed. If the liver is affected by the infection, bile ducts may become inflamed and trigger PSC. There’s also the possibility that the condition is linked with autoimmune disorders, and that the inflammation is caused by the body attacking healthy tissues due to problems with the immune system.

PSC is also strongly linked with IBD (inflammatory bowel disease), since 75% of people with PSC also have ulcerative colitis, a form of IBD. However, it’s not clear whether IBD causes PSC or vice versa, or if the conditions simply share potential causes, such as genetic traits, which means both are likely to occur at the same time.

How is Primary Sclerosing Cholangitis Treated?

There is no treatment to fix the damage that has already occurred to the bile ducts. The goal of treatment is to prevent any further damage and help alleviate current symptoms. For example, a variety of creams and ointments are prescribed to soothe itchy skin.

Vitamin supplements taken every day may help give the body needed nutrients that it can no longer naturally digest. Antibiotics may also be prescribed to help with infections in the bile ducts.

Worse cases may need endoscopic surgery to help get bile flowing through the bile ducts again. The very worst cases have livers that are so damaged that only a transplant can save them.

Primary Sclerosing Cholangitis Prevention

Unfortunately there is no known way to prevent PSC. However, individuals with the condition may be able to slow the deterioration of the liver and avoid complications of the disease by taking particularly good care of their liver.

Firstly, they should avoid alcohol completely. Although moderate amounts of alcohol are fine for healthy people, those with PSC tend are more susceptible to cirrhosis and other complications associated with alcohol use.

Secondly, it’s important to have vaccinations to protect against hepatitis A and B, both of which affect the liver. Since liver function and health is already compromised, contracting hepatitis A or B could dramatically worsen the condition of the liver.

Finally, be very careful when using chemicals, whether at home or at work. The liver is responsible for filtering out toxins in the blood, and regular exposure to harmful chemicals can damage it. For people with PSC, the effects of such chemicals can be even more severe. Wear appropriate protective equipment when handling chemicals, or avoid them altogether.

Last Reviewed:
October 09, 2016
Last Updated:
January 11, 2018