primary sclerosing cholangitis

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primary sclerosing cholangitis

idiopathic chronic hepatobiliary disease characterized by diffuse inflammation and fibrosis of the extrahepatic biliary system resulting in patchy, irregular stricturing of the bile ducts. Progressive bile duct obliteration to cirrhosis, hepatic failure, and bile duct cancer may occur in up to 50% of patients. Frequently associated with inflammatory bowel disease.

primary sclerosing cholangitis

a progressive chronic fibrosing inflammation of the bile ducts of unknown cause, occurring most commonly in young men and frequently in association with chronic ulcerative colitis. It also occurs as a complication of HIV infection. See also AIDS cholangiopathy.

primary sclerosing cholangitis

Sclerosing cholangitis A chronic idiopathic progressive liver disease characterized by inflammation, necrosis, fibrosis and obliteration of intrahepatic and extrahepatic bile ducts, resulting in cholestasis, cirrhosis, portal HTN, liver failure, often associated with other autoimmune diseases–eg, Crohn's disease, ulcerative colitis, Addison's disease Epidemiology PSC is uncommon–1 to 6/105, US; it is the 4th most common indication for liver transplant; 75% of Pts have IBD; 70% of Pts are ♂, average age 39 Clinical Often asymptomatic until end-stage liver disease; as albumin ↓ and BR ↑, pruritus, fatigue, jaundice, and weight loss dominate clinical picture, ± accompanied by fever, chills, night sweats, right upper quadrant pain Imaging Multiple zones of narrowing and dilatation– “beading” of bile duct by ERCP and transhepatic cholangiography Lab ↑ Alk phos, ↑ aminotransferase, hypergammaglobulinemia, ↑ IgM Management Azacytidine may ↓ need for transfusion, iron chelation–early use of deferiprone, corticosteroids, penicillamine, MTX; ursodiol is useless Prognosis Median survival after diagnosis, 12 yrs. See Primary biliary cirrhosis.

Primary sclerosing cholangitis

A chronic disease in which it is believed that the immune system fails to recognize the cells that compose the bile ducts as part of the same body, and attempts to destroy them. It is not clear what exactly causes the disease, but it is frequently associated with another inflammatory disease of the digestive tract, ulcerative colitis. The inflammation of the ducts eventually produces formation of scar tissue, causing multiple areas of narrowing (strictures) that block bile flow and lead to bacterial infection. Liver transplant gives the best chance for long-term survival.
Mentioned in: Cholangitis
References in periodicals archive ?
5) These cells in inflammatory lesions were initially detected in the pancreas (6) and are now known to be associated with multisystem IgG4-positive fibrosing disorders, including sclerosing cholangitis, sclerosing sialadenitis, and retroperitoneal fibrosis.