Primary Sclerosing Cholangitis: Epidemiology, Diagnosis, and Presentation

Clin Liver Dis. 2024 Feb;28(1):129-141. doi: 10.1016/j.cld.2023.07.005. Epub 2023 Aug 21.

Abstract

Primary sclerosing cholangitis (PSC) is considered an immunologically mediated disease. However, some of its features are not consistent with the typical profile of autoimmune conditions. PSC is characterized by progressive biliary fibrosis that may ultimately result in the eventual development of cirrhosis. In recent years, multiple studies have reported that the incidence and prevalence of this disease are on the rise. Consequently, patients are often diagnosed without symptoms or signs of advanced liver disease, although many still present with signs of decompensated liver disease. This article discusses the epidemiology, clinical presentation, and diagnostic workup in patients with PSC.

Keywords: Diagnosis; Epidemiology; Presentation; Primary sclerosing cholangitis.

Publication types

  • Review

MeSH terms

  • Autoimmune Diseases*
  • Cholangitis, Sclerosing* / diagnosis
  • Cholangitis, Sclerosing* / epidemiology
  • Humans
  • Liver Cirrhosis
  • Liver Diseases*