Cholangitis, sclerosing

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 and scared (sclerotic), and obstructed. This relentlessly progressive process can in time destroy the bile ducts and lead to cirrhosis. Primary sclerosing cholangitis (PSC) is also called idiopathic sclerosing cholangitis ("idiopathic" meaning the cause is not known). PSC can occur in an isolated form (by itself) or it can occur 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 are very much like those in PSC; however, in AIDS the changes in the biliary tract probably are due to infection and are not idiopathic. The infecting agents include mycoplasma and cytomegalovirus among others. 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. PSC is, in fact, one of the more common reasons for liver transplantation. The diagnosis of PSC is classically suspected on clinical (and routine laboratory) grounds and then confirmed by demonstration of thickened bile ducts using special radiologic tests called cholangiography. The treatment of PSC (short of liver transplantation) includes cholestyramine to diminish itching, antibiotics for infection, vitamin D and calcium to prevent bone loss (osteoporosis), and sometimes balloon dilatation or surgery for obstructed ducts. There is no medical treatment that has been demonstrated to slow the progression of PSC. The precise prognosis (outlook) depends on the age of the person, their degree of jaundice (based on the bilirubin level), the stage of their PSC on liver biopsy, and the size of the spleen (splenomegaly). Knowing these factors, one can predict the length of survival. Most patients die within 10 years of the time of diagnosis unless a liver transplant is performed.