TY - JOUR
T1 - Primary Sclerosing Cholangitis
AU - Larusso, Nicholas F.
AU - Wiesner, Russell H.
AU - Ludwig, Jurgen
AU - Maccarty, Robert L.
PY - 1984/4/5
Y1 - 1984/4/5
N2 - PRIMARY sclerosing cholangitis, an uncommonly diagnosed syndrome of unknown cause, is characterized by chronic fibrosing inflammation of bile ducts, usually affecting both the extrahepatic and intrahepatic biliary ductal systems.1,2 The disease leads to obliteration of intrahepatic bile ducts and to biliary cirrhosis. It can occur alone or in association with inflammatory bowel disease.3 It must be distinguished from bile-duct abnormalities resulting from biliary surgery, choledocholithiasis, bile-duct carcinoma, and congenital malformations. The course is generally one of slow progression to cirrhosis, portal hypertension, and death from liver failure; however, the time course of this progression is unpredictable. The syndrome is only.
AB - PRIMARY sclerosing cholangitis, an uncommonly diagnosed syndrome of unknown cause, is characterized by chronic fibrosing inflammation of bile ducts, usually affecting both the extrahepatic and intrahepatic biliary ductal systems.1,2 The disease leads to obliteration of intrahepatic bile ducts and to biliary cirrhosis. It can occur alone or in association with inflammatory bowel disease.3 It must be distinguished from bile-duct abnormalities resulting from biliary surgery, choledocholithiasis, bile-duct carcinoma, and congenital malformations. The course is generally one of slow progression to cirrhosis, portal hypertension, and death from liver failure; however, the time course of this progression is unpredictable. The syndrome is only.
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U2 - 10.1056/NEJM198404053101407
DO - 10.1056/NEJM198404053101407
M3 - Review article
C2 - 6366557
AN - SCOPUS:0021755499
SN - 0028-4793
VL - 310
SP - 899
EP - 903
JO - New England Journal of Medicine
JF - New England Journal of Medicine
IS - 14
ER -