Primary sclerosing cholangitis (PSC) is a chronic inflammatory disorder of the biliary system that results in progressive fibrosis and strictures of the intrahepatic and extrahepatic bile ducts. Typical radiological changes on retrograde cholangiography are shown in Figure 1. These strictures cause cholestatic liver disease that may progress to end-stage cirrhosis. The disorder has a strong ass...

BACKGROUND Primary sclerosing cholangitis is a rare chronic cholestatic condition of unknown etiology, frequently associated with inflammatory bowel disease and characterized by diffuse fibrosing and inflammatory destruction of the intra- and/or extrahepatic biliary duct system. PATIENTS AND METHODS The study involved 14 children with primary sclerosing cholangitis confirmed by either liver b...

The clinical features of 61 patients with sclerosing cholangitis were reviewed. This group included 23 patients with biliary tract calculi, commonly considered as excluding the diagnosis of primary sclerosing cholangitis. The aim of this study was to compare these 23 patients (group A) with 38 patients with sclerosing cholangitis free of calculi (group B). Both groups had the following features...

PRIMARY sclerosing cholangitis, stenosing cholangitis, fibrosing cholangitis, chronic obliterative cholangitis are all terms referring to a rare diffuse type ofchronic inflammation of the entire common bile duct and at times of the hepatic ducts and their radicles. In most instances, sclerosing cholangitis is idiopathic or primary. Bartholomew et al., in 1963 found only twenty unequivocal cases...

Primary sclerosing cholangitis (PSC) is a fibrosing disease of the intra- and extra-hepatic bile ducts, and is closely associated with inflammatory bowel disease. It is immune mediated, rather than being a classical autoimmune disease. A range of immune abnormalities have been demonstrated in PSC, in particular the findings of a range of autoantibodies, a portal tract infiltrate of functional T...

Primary sclerosing cholangitis (PSC) is an uncommon disorder of unknown etiology, characterized by chronic inflammation and fibrosis of the intra- and extrahepatic bile ducts. PSC is commonly associated with chronic inflammatory bowel disease, especially ulcerative colitis, and often in younger men with an extensive colitis. The diagnosis is made by endoscopic retrograde cholangiography. The ch...

Primary sclerosing cholangitis (PSC) is a generally progressive, sometimes fatal chronic hepatobiliary disorder for which no effective medical therapy now exists. This article describes the epidemiology of this disease, along with diagnosis and treatment options. Future research directions concerning PSC also are discussed.

Primary sclerosing cholangitis is the classic hepatobiliary manifestation of inflammatory bowel disease and is generally chronic and progressive. Patients frequently present with asymptomatic, anicteric cholestasis, but many develop progressive biliary strictures with time, leading to recurrent cholangitis, biliary cirrhosis, and end-stage liver disease. Medical treatment does not slow the prog...

A variety of diseases are included under the umbrella term 'cholangitis', including hepatobiliary diseases with an autoimmune pathogenesis (such as primary sclerosing cholangitis, primary biliary cholangitis, and IgG4-associated sclerosing cholangitis) and disease processes associated with intraductal stones and infectious etiologies (such as ascending bacterial cholangitis, recurrent pyogenic ...

Early studies in primary sclerosing cholangitis (PSC) were concerned with disease characterization, and were followed by epidemiological studies of PSC and clinical subsets of PSC as well as a large number of treatment trials. Recently, the molecular pathogenesis and the practical handling of the patients have received increasing attention. In the present review we aim to give an update on the ...