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Colangite esclerosante primária

Last reviewed: 9 Apr 2025
Last updated: 05 Mar 2025

Summary

Definition

History and exam

Key diagnostic factors

  • sexo masculino
  • história de doença inflamatória intestinal
Full details

Other diagnostic factors

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

Risk factors

  • sexo masculino
  • doença inflamatória intestinal (DII)
  • predisposição genética
Full details

Diagnostic tests

1st tests to order

  • 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
Full details

Tests to consider

  • 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
Full details

Treatment algorithm

ACUTE

doença inicial

doença hepática em estágio terminal

Contributors

Authors

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.

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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