The inconvenient truth of primary biliary cholangitis/autoimmune hepatitis overlap syndrome

Abstract

The term 'PBC/AIH-overlap' has been applied when features of autoimmune hepatitis (AIH), be they biochemical, serological or histological, coexist with primary biliary cholangitis (PBC), either at first presentation or sequentially during disease course. Several treatment paradigms have been proposed, extrapolated from those of the primary conditions. However, there are no randomised studies showing improved survival with combination therapy compared to bile acid monotherapy. In the absence of high-quality evidence, multidisciplinary patient-specific approaches must be used to individualise treatment pathways, with appreciation that disease phenotypes are not always static, differ in treatment responses, and have the potential to evolve over time.