PRIMARY SCLEROSING CHOLANGITIS

Primary sclerosing cholangitis, a chronic cholestatic liver disease of unknown cause, is characterized by ongoing inflammation, destruction, and fibrosis of intrahepatic and extrahepatic bile ducts.1–4 Over time, bile ducts become irregularly narrowed and obliterated, and small intrahepatic ducts disappear. Focal bile-duct dilatation proximal to areas of stricture produces a characteristic beaded appearance on cholangiography.5,6 Primary sclerosing cholangitis, although only about 1 percent as common as alcoholic liver disease,7,8 is the fourth leading indication for liver transplantation in adults in the United States.9 The disease progresses silently, but relentlessly, in most patients and leads to cirrhosis, portal hypertension,. © 1995, Massachusetts Medical Society. All rights reserved.