Primary sclerosing cholangitis: Epidemiology and pathogenesis
- Kris V Kowdley, MD, FACP, FACG, FASGE, AGAF
Kris V Kowdley, MD, FACP, FACG, FASGE, AGAF
- Clinical Professor of Medicine
- University of Washington School of Medicine
Primary sclerosing cholangitis (PSC) is a chronic cholestatic disease of the liver and bile ducts that is frequently progressive and can lead to end-stage liver disease. The disease is characterized by progressive inflammation, fibrosis, and stricturing of the intrahepatic and extrahepatic bile ducts (picture 1). The diagnosis is best established by contrast cholangiography, which reveals a characteristic picture of diffuse, multifocal strictures and focal dilation of the bile ducts, leading to a beaded appearance [1-3].
The term "primary" is used to distinguish PSC from other conditions that may lead to a similar clinical and cholangiographic syndrome . These include choledocholithiasis, bacterial cholangitis, prior biliary surgery, intra-arterial floxuridine, and acquired immunodeficiency syndrome associated with cholangiopathy .
The epidemiology and pathogenesis of PSC will be reviewed here. The clinical manifestations, diagnosis, and treatment of this disorder are discussed separately. (See "Primary sclerosing cholangitis in adults: Clinical manifestations and diagnosis" and "Primary sclerosing cholangitis in adults: Management".)
The epidemiology of PSC in the general population has not been well established. A systematic review that focused on eight studies (from North America and Europe) estimated that the overall incidence rate was 0.77 per 100,000 person-years, although there were substantial differences among individual studies . When focusing only on population-based studies, the incidence was estimated to be 1 per 100,000 person-years. The incidence was higher in males, and the median age at diagnosis was 41. All individual studies observed an overall increase in incidence during the time period examined.
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