Primary biliary cholangitis

Last reviewed: 29 Aug 2022
Last updated: 17 Dec 2019

Summary

Definition

History and exam

Key diagnostic factors

  • age 45-60 years
  • female sex
More key diagnostic factors

Other diagnostic factors

  • personal history of autoimmune disease
  • family history of autoimmune disease
  • history of hypercholesterolaemia
  • 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
Other diagnostic factors

Risk factors

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

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

Treatment algorithm

ACUTE

early-stage disease

developing end-stage liver disease or refractory pruritus

Contributors

Authors

David E. J. Jones, BA, BM, BCh, PhD, FRCP

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 reviewers

James Neuberger, BM, BCh

Consultant Physician

Liver Unit

Queen Elizabeth Hospital

Birmingham

UK

Disclosures

JN declares that he has no competing interests.

Ian R. Mackay, AM, MD, FAA, FRACP, FRCPA, FRCP

Department of Biochemistry and Molecular Biology

Monash University

Clayton

Victoria

Australia

Disclosures

IRM declares that he has no competing interests.

Ned Snyder, MD, FACP

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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