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Abstract

Primary sclerosing cholangitis (PSC) is a chronic cholestatic liver disease characterized by inflammation and fibrosis of the intra- and extrahepatic bile ducts. An estimated 80% of patients in North America and Europe have coexistent inflammatory bowel disease (IBD). The underlying pathophysiology of PSC remains poorly understood. As a result, there is currently no effective medical therapy to halt disease progression. Important complications from PSC include metabolic bone disease, colorectal neoplasia, and cholangiocarcinoma. Liver transplantation remains the only successful treatment option for patients with advanced liver disease from PSC. A diagnosis of PSC should be considered among individuals with IBD and elevated serum liver biochemical tests.