Primary sclerosing cholangitis

[Sarah]

Primary sclerosing cholangitis is inflammation with progressive scarring and narrowing of the bile ducts in and outside the liver. Eventually, the ducts become blocked and then obliterated. Cirrhosis, liver failure, and sometimes bile duct cancer develop.

Symptoms begin gradually and include worsening fatigue, itchiness, and, later, jaundice.
An imaging test can confirm the diagnosis.
Treatment focuses on relieving symptoms, but liver transplantation can prolong life.

Bile is a fluid that is produced by the liver and aids in digestion. Bile is transported through small tubes (bile ducts) that carry bile through the liver and then from the liver to the gallbladder and to the small intestine. (See also Overview of Gallbladder and Bile Duct Disorders.)
In primary sclerosing cholangitis, bile ducts become inflamed, which can lead to scarring of the bile ducts and liver tissue that progresses, eventually becoming severe (cirrhosis). The scar tissue narrows and blocks the bile ducts. As a result, bile salts, which help the body absorb fats, are not secreted normally. The disorder resembles primary biliary cholangitis except that it affects the bile ducts outside the liver as well as those in the liver. The cause is not known but is likely to be autoimmune (when the immune system attacks the body’s own tissues).
Primary sclerosing cholangitis most often affects young men and is diagnosed at an average age of 40. It commonly occurs in people with inflammatory bowel disease, especially ulcerative colitis. It tends to occur in families, suggesting that genes may be involved. An infection or injury of the bile ducts may trigger the disorder in people who have genes that make them susceptible to the disorder.

[Jessica]

chronic liver disease caused by inflammation and scarring of the bile ducts of the liver