Summary
Definition
History and exam
Key diagnostic factors
- age 40-65 years
- female sex
- family history of primary biliary cholangitis
Other diagnostic factors
- personal history of autoimmune disease
- family history of autoimmune disease
- history of hypercholesterolemia
- itch
- fatigue
- dry eyes and dry mouth
- abdominal discomfort
- sleep disturbance
- hepatomegaly
- xanthelasmata
- postural dizziness/blackouts
- memory and concentration problems
- jaundice
- ascites
- splenomegaly
- skin pigmentation
Risk factors
- female sex
- age between 40-65 years
- family history of PBC
- family history of autoimmune disease
- smoking
- urinary tract infection
Diagnostic investigations
1st investigations to order
- alkaline phosphatase (ALP)
- gamma-glutamyl transferase (GTT)
- bilirubin
- alanine aminotransferase (ALT)
- serum albumin
- antimitochondrial antibody (AMA) 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 (MRCP)
Investigations to consider
- serum immunoglobulin
- liver biopsy
- transient elastography
Treatment algorithm
early-stage disease
developing end-stage liver disease or refractory pruritus
Contributors
Authors
David Bernstein, MD
Professor of Medicine and Educational Science
Zucker School of Medicine at Hofstra/Northwell
Hempstead
Chief of the Division of Hepatology
Director of the Sandra Atlas Bass Center for Liver Diseases
Northwell Health
Manhasset
NY
Disclosures
DB is a consultant for OcelotBio, Abbvie, and Gilead. DB is on the speakers bureau for Intercept.
Acknowledgements
Dr David Bernstein would like to gratefully acknowledge Dr David E. J. Jones, the previous contributor to this topic. 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.
Alia S. Dadabhai, MD
Assistant Professor
Gastroenterology and Hepatology Division
Johns Hopkins University
Baltimore
ML
Disclosures
AD declares that she has no competing interests.
Differentials
- Obstructive bile duct lesion
- Small-duct primary sclerosing cholangitis
- Drug-induced cholestasis
More DifferentialsGuidelines
- EASL clinical practice guidelines on non-invasive tests for evaluation of liver disease severity and prognosis - 2021 update
- Primary biliary cholangitis: 2021 practice guidance update
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