Primary Scelerosing Cholangitis (PSC) is a disease that damages and blocks bile ducts inside and outside the liver. Bile is a liquid made in the liver. Bile ducts are tubes that carry bile out of the liver to the gallbladder and small intestine. In the intestine, bile helps break down fat in food.
In PSC, inflammation of the bile ducts leads to scar formation and narrowing of the ducts over time. As scarring increases, the ducts become blocked. As a result, bile builds up in the liver and damages liver cells. Eventually, scar tissue can spread throughout the liver, causing cirrhosis and liver failure.
What causes PSC?
The causes of PSC are not known. Genes, immune system problems, bacteria, and viruses may play roles in the development of the disease.
PSC is linked to inflammatory bowel disease (IBD). About three out of four people with PSC have a type of IBD called ulcerative colitis. The link between PSC and IBD is not yet understood.
Who gets PSC?
Most people with PSC are adults but the disease also occurs in children. The average age at diagnosis is 40. PSC is more common in men than women. Having family members with PSC may increase a person's risk for developing PSC.
What are the symptoms of PSC?
The main symptoms of PSC are itching, fatigue, and yellowing of the skin or whites of the eyes. An infection in the bile ducts can cause chills and fever. PSC progresses slowly, so a person can have the disease for years before symptoms develop.
What are the complications of PSC?
PSC can lead to various complications, including:
