Primary Biliary Cholangitis in Males: Pathogenesis, Clinical Presentation, and Prognosis

Clin Liver Dis. 2022 Nov;26(4):643-655. doi: 10.1016/j.cld.2022.06.008. Epub 2022 Sep 14.

Abstract

Primary biliary cholangitis (PBC) is an immune-mediated chronic liver disease characterized by progressive cholestasis, bile duct destruction, biliary fibrosis, and cirrhosis. Patients who respond to ursodeoxycholic acid have an expected survival similar to the general population. Although PBC primarily affects females, the prevalence in males is higher than was previously believed, with contemporary studies suggesting a female-to-male ratio of 4-6:1. A diagnosis of PBC is often delayed among males because of the myth that PBC is rare in males.

Keywords: Autoimmune hepatitis; Clinical presentation; Epidemiology; Men; Primary biliary cholangitis; Prognosis.

Publication types

  • Review

MeSH terms

  • Cholangitis* / diagnosis
  • Cholangitis* / drug therapy
  • Cholestasis*
  • Female
  • Humans
  • Liver Cirrhosis, Biliary* / diagnosis
  • Liver Cirrhosis, Biliary* / drug therapy
  • Liver Cirrhosis, Biliary* / epidemiology
  • Liver Diseases*
  • Male
  • Prognosis
  • Ursodeoxycholic Acid / therapeutic use

Substances

  • Ursodeoxycholic Acid