Last reviewed: 26 Jan 2021
Last updated: 08 May 2019



History and exam

Key diagnostic factors

  • male sex
  • history of inflammatory bowel disease

Other diagnostic factors

  • age in 40s or 50s
  • abdominal pain
  • pruritus
  • fatigue
  • weight loss
  • fever
  • jaundice
  • steatorrhoea
  • splenomegaly
  • ascites
  • encephalopathy

Risk factors

  • male sex
  • inflammatory bowel disease (IBD)
  • genetic predisposition

Diagnostic investigations

1st investigations to order

  • serum alkaline phosphatase
  • serum gamma-GT
  • serum aminotransferases (aspartate aminotransferase, alanine aminotransferase)
  • serum total bilirubin
  • serum albumin
  • FBC
  • prothrombin time
  • atypical antineutrophil cytoplasmic auto-antibody (ANCA)
  • antimitochondrial antibody
  • abdominal ultrasound
  • magnetic resonance cholangiopancreatography (MRCP)
  • endoscopic retrograde cholangiopancreatography (ERCP)

Investigations to consider

  • serum immunoglobulins
  • serum IgG4
  • hepatic copper concentration
  • urine copper concentration
  • ceruloplasmin
  • anti-nuclear antibody
  • anti-smooth muscle antibody
  • abdominal CT scan
  • liver biopsy
  • bone mineral density scan
  • colonoscopy

Treatment algorithm


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.

Peer reviewersVIEW ALL

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.

Consultant Physician

Liver Unit

Queen Elizabeth Hospital




JN declares that he has no competing interests.

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