Immunopathogenesis of Primary Biliary Cholangitis, Primary Sclerosing Cholangitis and Autoimmune Hepatitis: Themes and Concepts

Abstract

Autoimmune liver diseases (AILDs) include primary biliary cholangitis (PBC), primary sclerosing cholangitis (PSC) and autoimmune hepatitis (AIH), a family of chronic immune-mediated disorders that target hepatocytes and cholangiocytes. Treatments remain non-specific, variably effective and non-curative, and the need for liver transplantation is disproportionate to their rarity. Development of effective therapies requires better knowledge of pathogenic mechanisms, including the roles of genetic risk, and how the environment and gut dysbiosis cause immune cell dysfunction and aberrant bile acid signalling. This review summarises key aetiolopathogenic concepts and themes relevant for clinical practice, and how such learnings can guide the development of new therapies for people living with AILDs.