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.
Primary sclerosing cholangitis (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. It is often associated with inflammatory bowel disease. Complications include dominant biliary 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).
History and exam
- serum alkaline phosphatase
- serum gamma-GT
- serum aminotransferases (aspartate aminotransferase, alanine aminotransferase)
- serum total bilirubin
- serum albumin
- prothrombin time
- atypical antineutrophil cytoplasmic autoantibody (ANCA)
- antimitochondrial antibody
- abdominal ultrasound
- magnetic resonance cholangiopancreatography (MRCP)
- endoscopic retrograde cholangiopancreatography (ERCP)
S. Ian Gan, MD, FRCPC
Associate Clinical Professor
Vancouver General Hospital
University of British Columbia
SIG declares that he has no competing interests.
Dr S. Ian Gan would like to gratefully acknowledge Dr Kris V. Kowdley and Dr Christine Schlenker, the previous contributors to this topic.
KVK is a member of the speakers bureau of Axcan Pharma, manufacturer of Urso250 and Urso Forte, and gives one or two lectures a year on treatment of cholestatic liver diseases. KVK has also received funding from the NIH for a research study of Urso in PSC. CS declares that she has no competing interests.
Marlyn Mayo, MD
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.
James Neuberger, BM, BCh
Queen Elizabeth Hospital
JN declares that he has no competing interests.
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