Last reviewed: February 2020
Last updated: December  2019

Summary

Definition

History and exam

Other diagnostic factors

  • personal history of autoimmune disease
  • family history of autoimmune disease
  • history of hypercholesterolemia
  • itch
  • fatigue
  • dry eyes and dry mouth
  • sleep disturbance
  • postural dizziness/blackouts
  • hepatomegaly
  • family history¬†of primary biliary cholangitis
  • weight loss and other metabolic features of malabsorption
  • memory and concentration problems
  • jaundice
  • ascites
  • splenomegaly
  • skin pigmentation
  • xanthelasmata

Risk factors

  • female sex
  • age between 45-60 years
  • family history of PBC/autoimmune disease
  • smoking
  • urinary tract infection

Diagnostic investigations

1st investigations to order

  • alkaline phosphatase
  • gamma-GT
  • bilirubin
  • alanine aminotransferase (ALT)
  • serum albumin
  • antimitochondrial antibody immunofluorescence
  • antinuclear antibody (ANA) immunofluorescence
  • antipyruvate dehydrogenase complex-E2 ELISA
  • anti-M2 ELISA
  • antiglycoprotein-210 ELISA
  • anti-Sp100 ELISA
  • abdominal ultrasound scan
  • magnetic resonance cholangiopancreatography
More 1st investigations to order

Investigations to consider

  • prothrombin time
  • serum immunoglobulin
  • liver biopsy
  • upper GI endoscopy
  • serum alpha-fetoprotein
More investigations to consider

Emerging tests

  • transient elastography
More emerging tests

Treatment algorithm

Contributors

Professor of Liver Immunology

University of Newcastle

Honorary Consultant Hepatologist

Freeman Hospital

Newcastle-upon-Tyne

UK

Disclosures

DEJJ has received speaker honoraria from Falk, Intercept, and Abbott, grant funding from Intercept and Pfizer, and has undertaken consultancy work for Falk, GSK, Intercept, and Novartis. DEJJ is an author of a number of articles referenced in this topic.

Peer reviewersVIEW ALL

Consultant Physician

Liver Unit

Queen Elizabeth Hospital

Birmingham

UK

Disclosures

JN declares that he has no competing interests.

Department of Biochemistry and Molecular Biology

Monash University

Clayton

Victoria

Australia

Disclosures

IRM declares that he has no competing interests.

Professor of Medicine

Chief of Clinical Gastroenterology and Hepatology

University of Texas Medical Branch

Galveston

TX

Disclosures

NS declares that he has no competing interests.

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