Last reviewed: October 2017
Last updated: November  2017

Summary

Definition

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
  • steatorrhoea
  • 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
  • FBC
  • prothrombin time
  • atypical antineutrophil cytoplasmic auto-antibody (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

Contributors

Authors VIEW ALL

Director of Education

Digestive Disease Institute

Virginia Mason Medical Center

Seattle

WA

Disclosures

SIG declares that he has no competing interests.

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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