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 in these patients.
Chronic progressive cholestatic liver disease, characterised by inflammation and fibrosis of the intrahepatic and/or extrahepatic bile ducts, resulting in diffuse, multi-focal stricture formation. It is often associated with inflammatory bowel disease. Complications include dominant biliary stricture (a focal area of tight narrowing of the extrahepatic biliary tree that develops in 40% to 50% of patients as a result of progressive structuring) and end-stage liver disease (due to chronic progressive biliary fibrosis).
Director of Education
Digestive Disease Institute
Virginia Mason Medical Center
SIG declares that he has no competing interests.
Associate Professor of Medicine
Division of Digestive and Liver Diseases
University of Texas Southwestern Medical Center
MM is an author of a reference cited in this monograph.
Queen Elizabeth Hospital
JN declares that he has no competing interests.
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