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Primary sclerosing cholangitis

Last reviewed: 25 Jun 2024
Last updated: 14 Jul 2023

Summary

Definition

History and exam

Key diagnostic factors

  • male sex
  • history of inflammatory bowel disease
Full details

Other diagnostic factors

  • age 25 to 45 years
  • abdominal pain
  • pruritus
  • fatigue
  • weight loss
  • fever
  • jaundice
  • steatorrhea
  • splenomegaly
  • ascites
  • encephalopathy
Full details

Risk factors

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

Diagnostic tests

1st tests to order

  • serum alkaline phosphatase
  • serum gamma-GT
  • serum aminotransferases (aspartate aminotransferase, alanine aminotransferase)
  • serum total bilirubin
  • serum albumin
  • CBC
  • prothrombin time
  • abdominal ultrasound
  • magnetic resonance cholangiopancreatography (MRCP)
  • serum IgG4
Full details

Tests to consider

  • endoscopic retrograde cholangiopancreatography (ERCP)
  • serum immunoglobulins
  • 24 hour urine copper concentration
  • ceruloplasmin
  • antinuclear antibody
  • anti-smooth muscle antibody
  • antimitochondrial antibody
  • abdominal CT scan
  • liver biopsy
  • bone mineral density scan
  • colonoscopy
Full details

Treatment algorithm

ACUTE

early disease

end-stage liver disease

Contributors

Authors

S. Ian Gan, MD, FRCPC

Associate Clinical Professor

Vancouver General Hospital

University of British Columbia

Vancouver

BC

Disclosures

SIG declares that he has no competing interests.

Nawaf Tareq Aboalfaraj, MBBS, FRCPC

Gastroenterology Fellow

Vancouver General Hospital

University of British Columbia

Vancouver

BC

Disclosures

NTA declares that he has no competing interests.

Acknowledgements

Dr S. Ian Gan and Dr Nawaf Tareq Aboalfaraj would like to gratefully acknowledge Dr Kris V. Kowdley and Dr Christine Schlenker, the previous contributors to this topic.

Disclosures

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

Marlyn Mayo, MD

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 topic.

James Neuberger, BM, BCh

Consultant Physician

Liver Unit

Queen Elizabeth Hospital

Birmingham

UK

Disclosures

JN declares that he has no competing interests.

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  • Guidelines

    • ACR Appropriateness Criteria: abnormal liver function tests
    • EASL clinical practice guidelines on sclerosing cholangitis
    More Guidelines
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