Summary
Definition
History and exam
Key diagnostic factors
- male sex
- history of inflammatory bowel disease
Other diagnostic factors
- age in 40s or 50s
- abdominal pain
- pruritus
- fatigue
- weight loss
- fever
- jaundice
- steatorrhoea
- splenomegaly
- ascites
- encephalopathy
Risk factors
- male sex
- inflammatory bowel disease (IBD)
- genetic predisposition
Diagnostic investigations
1st investigations to order
- serum alkaline phosphatase
- serum gamma-GT
- serum aminotransferases (aspartate aminotransferase, alanine aminotransferase)
- serum total bilirubin
- serum albumin
- FBC
- prothrombin time
- atypical antineutrophil cytoplasmic auto-antibody (ANCA)
- antimitochondrial antibody
- abdominal ultrasound
- magnetic resonance cholangiopancreatography (MRCP)
- endoscopic retrograde cholangiopancreatography (ERCP)
Investigations to consider
- serum immunoglobulins
- serum IgG4
- hepatic copper concentration
- urine copper concentration
- ceruloplasmin
- anti-nuclear antibody
- anti-smooth muscle antibody
- abdominal CT scan
- liver biopsy
- bone mineral density scan
- colonoscopy
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.
Acknowledgements
Dr S. Ian Gan 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.
Differentials
- Secondary sclerosing cholangitis
- Immunoglobulin G4 cholangitis
- Autoimmune hepatitis
More DifferentialsGuidelines
- British Society of Gastroenterology and UK-PSC guidelines for the diagnosis and management of primary sclerosing cholangitis
- ACG clinical guideline: primary sclerosing cholangitis
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