Summary
Definition
History and exam
Key diagnostic factors
- male sex
- history of inflammatory bowel disease
Other diagnostic factors
- age 25 to 45 years
- abdominal pain
- pruritus
- fatigue
- weight loss
- fever
- jaundice
- steatorrhea
- splenomegaly
- ascites
- encephalopathy
Risk factors
- male sex
- inflammatory bowel disease (IBD)
- genetic predisposition
Diagnostic tests
1st tests to order
- serum alkaline phosphatase
- serum gamma-GT
- serum aminotransferases (aspartate aminotransferase, alanine aminotransferase)
- serum total bilirubin
- serum albumin
- CBC
- prothrombin time
- abdominal ultrasound
- magnetic resonance cholangiopancreatography (MRCP)
- serum IgG4
Tests to consider
- endoscopic retrograde cholangiopancreatography (ERCP)
- serum immunoglobulins
- 24 hour urine copper concentration
- ceruloplasmin
- antinuclear antibody
- anti-smooth muscle antibody
- antimitochondrial antibody
- abdominal CT scan
- liver biopsy
- bone mineral density scan
- colonoscopy
- transient elastography
- magnetic resonance elastography
Treatment algorithm
early disease
end-stage liver disease
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
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.
Differentials
- Secondary sclerosing cholangitis
- Immunoglobulin G4 cholangitis
- Autoimmune hepatitis
More DifferentialsGuidelines
- ACR Appropriateness Criteria: abnormal liver function tests
- EASL clinical practice guidelines on sclerosing cholangitis
More Guidelines
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