For support and advice from others, visit our Primary Sclerosing Cholangitis Community
Primary Sclerosing 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.
PSC damages the hepatic, cystic, and common bile ducts, which carry bile out of the liver.
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.
The causes of PSC are not known. Genes, immune
system problems, bacteria, and viruses may play roles in the development of the
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.
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.
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.
PSC can lead to various complications, including
Blood tests to check levels of liver enzymes are
the first step in diagnosing PSC. Doctors confirm the diagnosis using
cholangiography, which provides pictures of the bile ducts.
Cholangiography can be performed in the following ways:
Other testing may include ultrasound exams and a liver biopsy. Ultrasound uses sound waves to create images of organs inside the body. A biopsy involves removal of a small piece of tissue for examination with a microscope.
Although researchers have studied many treatments,
none has been shown to cure or slow the progress of PSC. Treatment of PSC aims
to relieve symptoms and manage complications. Medical treatment may include
various medications to relieve itching, antibiotics to treat infections, and
vitamin supplements. Instruments passed through an endoscope during ERCP can
help open blocked bile ducts.
Liver transplantation may be an option if the liver begins to fail.
The National Institute of Diabetes and Digestive
and Kidney Diseases conducts and supports research related to digestive
diseases, including PSC.
Source: Information provided courtesy of the National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK), a division of the National Institutes of Health (NIH).
The National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK) is a division of the National Institutes of Health (NIH) of the U.S. Department of Health and Human Services. Established in 1980, the Clearinghouse provides information about digestive diseases to people with digestive disorders and to their families, health care professionals, and the public.