Biliary tract inflammatory disorders: Primary sclerosing cholangitis and primary biliary cirrhosis |
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Institution: | (1) Medical College of Wisconsin, 9200 West Wisconsin Avenue, 53226 Milwaukee, WI, USA |
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Abstract: | Primary sclerosing cholangitis (PSC) and primary biliary cirrhosis (PBC) are chronic progressive cholestatic diseases that
frequently lead to biliary cirrhosis. The exact pathogenesis of these diseases remains elusive but is likely immunologically
based. Complications range from fatigue and pruritus to end-stage liver disease. The risk of developing hepatocellular carcinoma
is low for patients with PBC, whereas cholangiocarcinoma is common in PSC and carries an ominous prognosis. Although ursodeoxycholic
acid is effective in slowing the progression of PBC, no effective medical therapy exists for PSC. Liver transplantation is
the only option for patients with endstage liver disease and yields excellent long-term survival in both groups. |
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