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Colangite esclerosante primária

  • Visão geral
  • Teoria
  • Diagnóstico
  • Tratamento
  • ACOMPANHAMENTO
  • Recursos
Última revisão: 13 Sep 2025
Última atualização: 05 Mar 2025

Resumo

Definição

História e exame físico

Principais fatores diagnósticos

  • sexo masculino
  • história de doença inflamatória intestinal
Detalhes completos

Outros fatores diagnósticos

  • idade entre 25 e 45 anos
  • dor abdominal
  • prurido
  • fadiga
  • perda de peso
  • febre
  • icterícia
  • esteatorreia
  • esplenomegalia
  • ascite
  • encefalopatia
Detalhes completos

Fatores de risco

  • sexo masculino
  • doença inflamatória intestinal (DII)
  • predisposição genética
Detalhes completos

Investigações diagnósticas

Primeiras investigações a serem solicitadas

  • fosfatase alcalina sérica
  • gama-glutamiltransferase sérica
  • aminotransferases séricas (aspartato aminotransferase, alanina aminotransferase)
  • bilirrubina total sérica
  • albumina sérica
  • Hemograma completo
  • tempo de protrombina
  • ultrassonografia abdominal
  • colangiopancreatografia por ressonância magnética (CPRM)
  • imunoglobulina G4 (IgG4) sérica
Detalhes completos

Investigações a serem consideradas

  • colangiopancreatografia retrógrada endoscópica (CPRE)
  • imunoglobulinas séricas
  • concentração de cobre urinário de 24 horas
  • ceruloplasmina
  • anticorpo antinuclear
  • anticorpo antimúsculo liso
  • anticorpo antimitocondrial
  • tomografia computadorizada (TC) abdominal
  • biópsia hepática
  • densitometria óssea
  • colonoscopia
  • elastografia transitória
  • elastografia por ressonância magnética
Detalhes completos

Algoritmo de tratamento

AGUDA

doença inicial

doença hepática em estágio terminal

Colaboradores

Autores

S. Ian Gan, MD, FRCPC

Associate Clinical Professor

Vancouver General Hospital

University of British Columbia

Vancouver

BC

Disclosures

SIG declares that he has no competing interests.

Nawaf Tareq Aboalfaraj, MBBS, FRCPC

Gastroenterology Fellow

Vancouver General Hospital

University of British Columbia

Vancouver

BC

Disclosures

NTA declares that he has no competing interests.

Acknowledgements

Dr S. Ian Gan and Dr Nawaf Tareq Aboalfaraj would like to gratefully acknowledge Dr Kris V. Kowdley and Dr Christine Schlenker, the previous contributors to this topic.

Disclosures

KVK is a member of the speakers bureau of Axcan Pharma, manufacturer of Urso250 and Urso Forte, and gives one or two lectures a year on treatment of cholestatic liver diseases. KVK has also received funding from the NIH for a research study of Urso in PSC. CS declares that she has no competing interests.

Peer reviewers

Marlyn Mayo, MD

Associate Professor of Medicine

Division of Digestive and Liver Diseases

University of Texas Southwestern Medical Center

Dallas

TX

Disclosures

MM is an author of a reference cited in this topic.

James Neuberger, BM, BCh

Consultant Physician

Liver Unit

Queen Elizabeth Hospital

Birmingham

UK

Disclosures

JN declares that he has no competing interests.

Peer reviewer acknowledgements

BMJ Best Practice topics are updated on a rolling basis in line with developments in evidence and guidance. The peer reviewers listed here have reviewed the content at least once during the history of the topic.

Disclosures

Peer reviewer affiliations and disclosures pertain to the time of the review.

References

Our in-house evidence and editorial teams collaborate with international expert contributors and peer reviewers to ensure that we provide access to the most clinically relevant information possible.

Key articles

LaRusso NF, Shneider BL, Black D, et al. Primary sclerosing cholangitis: summary of a workshop. Hepatology. 2006 Sep;44(3):746-64. Abstract

European Association for the Study of the Liver. EASL clinical practice guidelines on sclerosing cholangitis. J Hepatol. 2022 Sep;77(3):761-806.Full text  Abstract

Bowlus CL, Arrivé L, Bergquist A, et al. AASLD practice guidance on primary sclerosing cholangitis and cholangiocarcinoma. Hepatology. 2023 Feb 1;77(2):659-702.Full text  Abstract

Chapman R, Fevery J, Kalloo A, et al. Diagnosis and management of primary sclerosing cholangitis. Hepatology. 2010 Feb;51(2):660-78.Full text  Abstract

Dave M, Elmunzer BJ, Dwamena BA, et al. Primary sclerosing cholangitis: meta-analysis of diagnostic performance of MR cholangiopancreatography. Radiology. 2010 Aug;256(2):387-96. Abstract

Poropat G, Giljaca V, Stimac D, et al. Bile acids for primary sclerosing cholangitis. Cochrane Database Syst Rev. 2011 Jan 19;(1):CD003626.Full text  Abstract

Reference articles

A full list of sources referenced in this topic is available to users with access to all of BMJ Best Practice.
  • Colangite esclerosante primária images

  • Differentials

    • Colangite esclerosante secundária
    • Colangite relacionada à imunoglobulina G4 (IgG4)
    • Hepatite autoimune
    More Differentials

  • Guidelines

    • ACR Appropriateness Criteria: abnormal liver function tests
    • AASLD practice guidance on primary sclerosing cholangitis and cholangiocarcinoma
    More Guidelines

  • Calculators

    Escore MELDNa (para fins de listagem de transplantes de fígado, não é adequado para pacientes com menos de 12 anos de idade ) (unidades SI)

    More Calculators
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