Primary sclerosing cholangitis

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Last reviewed: May 2019

Last updated: May 2019

Summary

Cholestatic liver disease causing continued destruction of the bile ducts, cirrhosis, and end-stage liver disease.

Predominantly affects young and middle-aged men, often with underlying inflammatory bowel disease.

Patients may be asymptomatic at diagnosis but develop symptoms of pruritus and jaundice.

Diagnosis involves laboratory tests and cholangiography.

No effective medical therapy is available.

Liver transplantation is the only treatment option for patients with advanced disease.

Liver failure and cholangiocarcinoma are the leading causes of death.

Definition

Primary sclerosing cholangitis (PSC) is a chronic progressive cholestatic liver disease, characterized by inflammation and fibrosis of the intrahepatic and/or extrahepatic bile ducts, resulting in diffuse, multifocal stricture formation. It is often associated with inflammatory bowel disease. Complications include dominant biliary strictures (focal areas of tight narrowing of the extrahepatic biliary tree that develops in 40% to 50% of patients as a result of progressive structuring), cholangitis, cholangiocarcinoma, and end-stage liver disease (due to chronic progressive biliary fibrosis).

History and exam

Key diagnostic factors

male sex
history of inflammatory bowel disease

Full details

Other diagnostic factors

age in 40s or 50s
abdominal pain
pruritus
fatigue
weight loss
fever
jaundice
steatorrhea
splenomegaly
ascites
encephalopathy

Full details

Risk factors

male sex
inflammatory bowel disease (IBD)
genetic predisposition

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Diagnostic investigations

1st investigations to order

serum alkaline phosphatase
serum gamma-GT
serum aminotransferases (aspartate aminotransferase, alanine aminotransferase)
serum total bilirubin
serum albumin
CBC
prothrombin time
atypical antineutrophil cytoplasmic autoantibody (ANCA)
antimitochondrial antibody
abdominal ultrasound
magnetic resonance cholangiopancreatography (MRCP)
endoscopic retrograde cholangiopancreatography (ERCP)

Full details

Investigations to consider

serum immunoglobulins
serum IgG4
hepatic copper concentration
urine copper concentration
ceruloplasmin
antinuclear antibody
anti-smooth muscle antibody
abdominal CT scan
liver biopsy
bone mineral density scan
colonoscopy

Full details

Treatment algorithm

ACUTE

early disease

end-stage liver disease

Contributors

Authors VIEW ALL 

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.

Peer reviewers VIEW ALL 

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 monograph.

James Neuberger, BM, BCh

Consultant Physician

Liver Unit

Queen Elizabeth Hospital

Birmingham

UK

Disclosures

JN declares that he has no competing interests.

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