Last reviewed: November 2017
Last updated: November  2017

Summary

Definition

History and exam

Key diagnostic factors

  • age between 45 and 60 years
  • female sex

Other diagnostic factors

  • personal hx of autoimmune disease
  • FHx of autoimmune disease
  • hx of hypercholesterolaemia
  • itch
  • fatigue
  • dry eyes and dry mouth
  • sleep disturbance
  • postural dizziness/blackouts
  • hepatomegaly
  • FHx 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 and 60 years
  • FHx of PBC/autoimmune disease
  • smoking
  • urinary tract infection

Diagnostic investigations

1st investigations to order

  • alkaline phosphatase
  • gamma-GT
  • bilirubin
  • alanine aminotransferase
  • serum albumin
  • antimitochondrial antibody immunofluorescence
  • ANA immunofluorescence
  • antipyruvate dehydrogenase complex-E2 ELISA
  • anti-M2 ELISA
  • antiglycoprotein-210 ELISA
  • anti-Sp100 ELISA
  • abdominal ultrasound scan
Full details

Investigations to consider

  • prothrombin time
  • serum immunoglobulin
  • liver biopsy
  • upper GI endoscopy
  • serum alpha-fetoprotein
Full details

Emerging tests

  • transient elastography
Full details

Treatment algorithm

Contributors

Authors VIEW ALL

Professor of Liver Immunology

University of Newcastle

Honorary Consultant Hepatologist

Freeman Hospital

Newcastle-upon-Tyne

UK

Disclosures

DEJJ has received speaker honoraria from Falk, 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 monograph.

Peer reviewers VIEW 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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