Definition of Primary sclerosing cholangitis
Primary sclerosing cholangitis: 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.
Last Editorial Review: 4/5/2003
- colchicine, Colchicine - Read about colchicine, a drug prescribed for the pain of gouty arthritis. Side effects, drug interactions, dosage, and preagnancy information is also included in the information.
- Liver Blood Tests - Learn about liver blood tests used to detect liver damage disease such as fatty liver, cirrhosis, hepatitis, Tylenol liver damage, and more. This includes measuring the aminotransferases enzymes (AST and ALT levels)
- Abdominal Pain - Learn about abdominal pain (pain in the stomach / abdomen) including causes, symptoms, how abdominal pain is diagnosed, and how abdominal pain is treated.
Latest Medical News
Back to MedTerms online medical dictionary A-Z List
