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Sclerosing cholangitis.

Biliary tract

Current

Opinion in Gastroenterology. 23(3):310-316, May 2007.

Maggs, RL; Chapman, W

Abstract:

Purpose of review: Primary sclerosing cholangitis is a chronic cholestatic

liver disease characterized by strictures of the biliary tree complicated by

cirrhosis and cholangiocarcinoma. It is immune

mediated, although the precise aetiology remains

unknown.

Recent findings: Research into etiopathogenesis and epidemiology, diagnosis of

cholangiocarcinoma, associations with inflammatory bowel disease and autoimmune

pancreatitis, and medical therapy are discussed.

Summary: Multiple gene

polymorphisms associated with primary sclerosing cholangitis have been investigated. Common

inflammatory bowel disease-associated polymorphisms do not confer any

susceptibility to primary sclerosing cholangitis; the role of intercellular adhesion molecule-1

gene polymorphisms and CCR5 mutations remain unclear. Elevated

IgG4 has been demonstrated in a subgroup of primary sclerosing

cholangitis patients, which may indicate an overlap

with autoimmune pancreatitis and possible

responsiveness to steroids. Biliary brush cytology may

assist in diagnosis of cholangiocarcinoma, although further clinical indicators

are required. Animal studies suggest the superiority

of 24-norursodeoxycholic acid over ursodeoxycholic

acid in reducing histological disease progress; translational studies in humans

are now required.

© 2007 Lippincott

& Wilkins, Inc.

Barb in Texas - Together in the Fight, Whatever it Takes!

Son Ken (32) UC 91 - PSC 99 Listed 7/21 @ Baylor Dallas

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