Last reviewed: March 2019
Last updated: November  2017



History and exam

Key diagnostic factors

  • male gender
  • hx of inflammatory bowel disease

Other diagnostic factors

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

Risk factors

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

Diagnostic investigations

1st investigations to order

  • serum alkaline phosphatase
  • serum gamma-GT
  • serum aminotransferases (AST, ALT)
  • serum total bilirubin
  • serum albumin
  • CBC
  • prothrombin time
  • atypical antineutrophil cytoplasmic autoantibody (ANCA)
  • antimitochondrial antibody
  • abdominal ultrasound
  • MRCP
  • ERCP
Full details

Investigations to consider

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

Treatment algorithm


Authors VIEW ALL

Director of Education

Digestive Disease Institute

Virginia Mason Medical Center




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 monograph. 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 reviewers VIEW 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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