Primary biliary cholangitis

Visão geral 
Teoria 
Diagnóstico 
Tratamento 
ACOMPANHAMENTO 
Recursos 

Conectar-se ou assinar para acessar todo o BMJ Best Practice

Última revisão das evidências: 17 Jul 2026

Última atualização do tópico: 11 Aug 2026

Primary biliary cholangitis (PBC) is characterized by progressive intrahepatic bile duct damage and loss. Although usually progressive, in many people the rate of progression can be so slow that it may not be clinically relevant.

The combination of a cholestatic pattern of serum liver tests (elevated alkaline phosphatase, gamma-glutamyl transferase, or both) and a PBC-specific autoantibody (typically antimitochondrial antibody) is sufficient for diagnosis in most patients, with no need for biopsy confirmation.

Symptoms (typically pruritus and fatigue) can significantly lower the quality of life, even in patients with a very slowly progressive disease. Appropriate symptomatic management is therefore important.

Progression of the disease can be slowed by therapy with ursodiol. Second-line options include elafibranor and seladelpar. To a significant degree the treatments that modify progression of the disease do not modify symptoms (and vice versa).

Transplantation is an effective treatment for patients who develop end-stage liver disease with PBC.

Definição

PBC is a chronic disease of the small intrahepatic bile ducts that is characterized by progressive bile duct damage (and eventual loss) occurring in the context of chronic portal tract inflammation. Fibrosis develops as a consequence of the original insult and the secondary effects of toxic bile acids retained in the liver, resulting ultimately in cirrhosis. The almost universal presence of autoantibodies (classically antimitochondrial antibodies) has led to the widely held view that PBC is an autoimmune disease.[1] PBC is significantly more common in women than in men. Peak incidence is around age 40 years, and median age at diagnosis is 65 years.[2][3]

História e exame físico

Principais fatores diagnósticos

age 40-65 years
female sex
family history of primary biliary cholangitis (PBC)

Detalhes completos

Outros fatores diagnósticos

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

Detalhes completos

Fatores de risco

female sex
age between 40 and 65 years
family history of primary biliary cholangitis (PBC)
family history of autoimmune disease
smoking
urinary tract infection

Detalhes completos

Investigações diagnósticas

Primeiras investigações a serem solicitadas

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)
transient elastography

Detalhes completos

Investigações a serem consideradas

serum immunoglobulin
liver biopsy

Detalhes completos

Algoritmo de tratamento

AGUDA

early-stage disease

developing end-stage liver disease or refractory pruritus

Colaboradores

Autores

David Bernstein, MD

Professor of Medicine

NYU Grossman School of Medicine

Director, Gastroenterology and Hepatology

Ambulatory Network-Long Island

NYU Langone Health

New York

Declarações

DB is a consultant for Ipsen. DB is on the speakers bureau for Ipsen and Intercept.

Agradecimentos

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.

Revisores

James Neuberger, BM, BCh

Consultant Physician

Liver Unit

Queen Elizabeth Hospital

Birmingham

Declarações

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

Declarações

IRM declares that he has no competing interests.

Alia S. Dadabhai, MD

Assistant Professor

Gastroenterology and Hepatology Division

Johns Hopkins University

Baltimore

Declarações

AD declares that she has no competing interests.

Créditos aos pareceristas

Os tópicos do BMJ Best Practice são constantemente atualizados, seguindo os desenvolvimentos das evidências e das diretrizes. Os pareceristas aqui listados revisaram o conteúdo pelo menos uma vez durante a história do tópico.

Declarações

As afiliações e declarações dos pareceristas referem--se ao momento da revisão.

Referências

Nossas equipes internas de editoria e de evidências trabalham em conjunto com colaboradores internacionais especializados e pares revisores para garantir que forneçamos acesso às informações o mais clinicamente relevantes possível.

Principais artigos

Hirschfield GM, Dyson JK, Alexander GJM, et al. The British Society of Gastroenterology/UK-PBC primary biliary cholangitis treatment and management guidelines. Gut. 2018 Sep;67(9):1568-94.Texto completo Resumo

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.Texto completo Resumo

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. Resumo

European Association for the Study of the Liver. EASL clinical practice guidelines on non-invasive tests for evaluation of liver disease severity and prognosis - 2021 update. J Hepatol. 2021 Sep;75(3):659-89.Texto completo Resumo

Artigos de referência

Uma lista completa das fontes referenciadas neste tópico está disponível para os usuários com acesso total ao BMJ Best Practice.

Diagnósticos diferenciais
- Obstructive bile duct lesion
- Small-duct primary sclerosing cholangitis
- Drug-induced cholestasis
Mais Diagnósticos diferenciais
Diretrizes
- AASLD practice guideline on imaging-based non-invasive liver disease assessments of hepatic fibrosis and steatosis
- EASL clinical practice guidelines on non-invasive tests for evaluation of liver disease severity and prognosis - 2021 update
Mais Diretrizes
Conectar-se ou assinar para acessar todo o BMJ Best Practice

O uso deste conteúdo está sujeito ao nosso aviso legal

Primary biliary cholangitis

Resumo

Definição

História e exame físico

Principais fatores diagnósticos

Outros fatores diagnósticos

Fatores de risco

Investigações diagnósticas

Primeiras investigações a serem solicitadas

Investigações a serem consideradas

Algoritmo de tratamento

early-stage disease

developing end-stage liver disease or refractory pruritus

Colaboradores

Autores

David Bernstein, MD

Declarações

Agradecimentos

Revisores

James Neuberger, BM, BCh

Declarações

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

Declarações

Alia S. Dadabhai, MD

Declarações

Créditos aos pareceristas

Declarações

Referências

Principais artigos

Artigos de referência

Uma lista completa das fontes referenciadas neste tópico está disponível para os usuários com acesso total ao BMJ Best Practice.

Diagnósticos diferenciais

Diretrizes

Conectar-se ou assinar para acessar todo o BMJ Best Practice

Conectar-se ou assinar para acessar todo o BMJ Best Practice

Conectar-se para acessar todo o BMJ Best Practice