Overlap Syndrome of Autoimmune Hepatitis and Primary Biliary Cholangitis

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.