Primary sclerosing cholangitis

Primary sclerosing cholangitis (PSC) is a cholestatic liver disease causing continued destruction of the bile ducts, cirrhosis, and end-stage liver disease.

Predominantly affects young and middle-aged men, often with underlying inflammatory bowel disease.

Patients may be asymptomatic at diagnosis but develop symptoms of pruritus and jaundice.

Diagnosis involves laboratory tests and cholangiography.

No effective medical therapy is available.

Liver transplantation is the only treatment option for patients with advanced disease.

Liver failure and cholangiocarcinoma are the leading causes of death.

PSC is a chronic progressive cholestatic liver disease, characterized by inflammation and fibrosis of the intrahepatic and/or extrahepatic bile ducts, resulting in diffuse, multifocal stricture formation. The more common, "classic" PSC, involves the large ducts (intra- and extrahepatic ducts that can be seen by cholangiography), the less common variant affects only the small bile ducts (bile ducts appear normal on cholangiography).

PSC is often associated with inflammatory bowel disease. A small percentage of patients (more often children) have clinical, biochemical, and histologic features of autoimmune hepatitis (PSC‐autoimmune hepatitis overlap syndrome). Complications of PSC include dominant/relevant strictures (focal areas of tight narrowing of the extrahepatic biliary tree that develops in 40% to 50% of patients as a result of progressive structuring), cholangitis, cholangiocarcinoma, and end-stage liver disease (due to chronic progressive biliary fibrosis).