Last reviewed: April 2019
Last updated: May  2019

Summary

Definition

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)
Full details

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
Full details

Treatment algorithm

Contributors

Authors VIEW ALL

Associate Clinical Professor

Vancouver General Hospital

University of British Columbia

Vancouver

BC

Disclosures

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.

Peer reviewers VIEW ALL

Associate Professor of Medicine

Division of Digestive and Liver Diseases

University of Texas Southwestern Medical Center

Dallas

TX

Disclosures

MM is an author of a reference cited in this monograph.

Consultant Physician

Liver Unit

Queen Elizabeth Hospital

Birmingham

UK

Disclosures

JN declares that he has no competing interests.

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