Primary sclerosing cholangitis

<B>Primary sclerosing cholangitis:</B> A chronic disorder of the liver in which the ducts carrying bile from the liver to the intestine, and often the ducts carrying bile within the liver, become inflamed, thickened, scarred (sclerotic), and obstructed.

This relentlessly progressive process can in time destroy the bile ducts and lead to cirrhosis.

Primary sclerosing cholangitis (PSC) can occur by itself or in association with other diseases, including inflammatory bowel disease, especially with ulcerative colitis; certain uncommon diseases, such as multifocal fibrosclerosis syndrome, Riedel's struma, and pseudotumor of the orbit; and AIDS.

Changes in the biliary tract are quite common in AIDS and very similar to those in PSC; however, in AIDS the changes in the biliary tract are probably due to infection with mycoplasma, cytomegalovirus, or other agents.

PSC often triggers jaundice (yellowing), pruritus (generalized itching all over the body), upper abdominal pain, and infection.

Later on, PSC progresses to cirrhosis of the liver and liver failure, creating a need for liver transplantation.

Diagnosis is by clinical observation and routine laboratory tests, and is confirmed by demonstration of thickened bile ducts using special radiologic tests called cholangiography.

Treatment includes cholestyramine to diminish itching, antibiotics for infection, vitamin D and calcium to prevent bone loss (osteoporosis), sometimes balloon dilatation or surgery for obstructed ducts, and liver transplantation when necessary and possible.

Prognosis depends on the age of the person, degree of jaundice, the stage of PSC found via liver biopsy, and the size of the spleen.

Most patients die within 10 years of diagnosis unless a liver transplant is performed.

Also known as idiopathic sclerosing cholangitis.