Summary
Definition
History and exam
Key diagnostic factors
- age 45-60 years
- female sex
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
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
Investigations to consider
- prothrombin time
- serum immunoglobulin
- liver biopsy
- upper GI endoscopy
- serum alpha-fetoprotein
Emerging tests
- transient elastography
Treatment algorithm
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.
Differentials
- Obstructive bile duct lesion
- Small-duct primary sclerosing cholangitis
- Drug-induced cholestasis
More DifferentialsGuidelines
- Primary biliary cholangitis
- The diagnosis and management of patients with primary biliary cholangitis
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