Summary
Definition
History and exam
Key diagnostic factors
- idade 40-65 anos
- sexo feminino
- história familiar de CBP
Other diagnostic factors
- história pessoal de doença autoimune
- história familiar de doença autoimune
- história de hipercolesterolemia
- prurido
- fadiga
- ressecamento dos olhos e da boca
- desconforto abdominal
- perturbação do sono
- hepatomegalia
- xantelasma
- tontura postural/blackouts
- problemas de memória e concentração
- icterícia
- ascite
- esplenomegalia
- pigmentação da pele
Risk factors
- sexo feminino
- idade entre 40 e 65 anos
- história familiar de CBP
- história familiar de doença autoimune
- tabagismo
- infecção do trato urinário
Diagnostic investigations
1st investigations to order
- fosfatase alcalina (FAL)
- gama-glutamiltransferase (GGT)
- bilirrubina
- alanina aminotransferase (ALT)
- albumina sérica
- imunofluorescência de anticorpo antimitocondrial (AAM)
- imunofluorescência de fator antinuclear (FAN)
- ELISA para o complexo E2 antipiruvato desidrogenase
- ELISA para anti-M2
- ELISA para antiglicoproteína-210
- ELISA para anti-Sp100
- ultrassonografia abdominal
- colangiopancreatografia por ressonância magnética (CPRM)
- elastografia transitória
Investigations to consider
- imunoglobulina sérica
- biópsia hepática
Treatment algorithm
doença em estágio inicial
doença hepática em estádio terminal em evolução ou prurido refratário
Contributors
Authors
David Bernstein, MD
Professor of Medicine
NYU Grossman School of Medicine
Director, Gastroenterology and Hepatology
Ambulatory Network-Long Island
NYU Langone Health
New York
NY
Disclosures
DB is a consultant for Ipsen. DB is on the speakers bureau for Ipsen and 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.
References
Key articles
Lindor KD, Bowlus CL, Boyer J, et al. Primary biliary cholangitis: 2018 practice guidance from the American Association for the Study of Liver Diseases. Hepatology. 2019 Jan;69(1):394-419.Full text Abstract
European Association for the Study of the Liver. EASL clinical practice guidelines: the diagnosis and management of patients with primary biliary cholangitis. J Hepatol. 2017 Jul;67(1):145-72. Abstract
Reference articles
A full list of sources referenced in this topic is available to users with access to all of BMJ Best Practice.
Differentials
- Lesão obstrutiva do ducto biliar
- Colangite esclerosante primária de pequenos ductos
- Colestase induzida por medicamento
More DifferentialsGuidelines
- AASLD practice guideline on imaging-based non-invasive liver disease assessments of hepatic fibrosis and steatosis
- Primary biliary cholangitis: 2021 practice guidance update
More Guidelines
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