Overlap Syndrome of Autoimmune Hepatitis and Primary Biliary Cholangitis

Clin Liver Dis. 2018 Aug;22(3):603-611. doi: 10.1016/j.cld.2018.03.010.

Abstract

Overlap syndrome of autoimmune hepatitis (AIH) and primary biliary cholangitis (PBC) is typically defined as concomitant or serial presentation with clinical features of both of these 2 distinct diseases. The Paris criteria and variations of the International Autoimmune Hepatitis group scoring systems for the diagnosis of AIH have been used to diagnose overlap syndrome. If left untreated, patients with overlap syndrome will have higher rates of portal hypertension, gastrointestinal bleeding, ascites, death, and need for liver transplant. Therefore, early identification is essential in providing appropriate therapy to potentially prevent long-term adverse outcomes in patients with overlap syndrome.

Keywords: Autoimmune hepatitis; Overlap syndrome; Primary biliary cholangitis.

Publication types

  • Review

MeSH terms

  • Ascites / etiology
  • Cholagogues and Choleretics / therapeutic use*
  • Drug Therapy, Combination
  • Esophageal and Gastric Varices / etiology
  • Gastrointestinal Hemorrhage / etiology
  • Glucocorticoids / therapeutic use
  • Hepatitis, Autoimmune / complications
  • Hepatitis, Autoimmune / drug therapy*
  • Humans
  • Hypertension, Portal / etiology
  • Immunosuppressive Agents / therapeutic use*
  • Liver Cirrhosis, Biliary / complications
  • Liver Cirrhosis, Biliary / drug therapy*
  • Liver Transplantation / statistics & numerical data
  • Mortality
  • Ursodeoxycholic Acid / therapeutic use

Substances

  • Cholagogues and Choleretics
  • Glucocorticoids
  • Immunosuppressive Agents
  • Ursodeoxycholic Acid