Clinical Immunology (Sixth Edition), Chapter 76 - Inflammatory Hepatobiliary Diseases

Typical histological findings of autoimmune hepatitis showing marked lymphoplasmacellular infiltration of the portal tract with interface hepatitis.
Elsevier, Clinical Immunology (Sixth Edition) Principles and Practice 2023, Pages 972-982
Benedetta Terziroli Beretta-Piccoli, Carlo Selmi, Michael P. Manns, M. Eric Gershwin.

Inflammatory hepatobiliary disease refers to chronic autoimmune diseases with predominant hepatic or biliary manifestations, without evidence of infectious diseases. Based on the target tissue, we can distinguish autoimmune hepatitis (AIH), targeting hepatocytes, from primary biliary cholangitis (PBC) and primary sclerosing cholangitis (PSC), both targeting the biliary tract. Cirrhosis and eventually liver failure are the common evolution of inflammatory hepatobiliary diseases, regardless of the target tissue, despite pathogenesis and therapeutics varying in between the spectrum of inflammatory hepatobiliary diseases. In this chapter we aim at elucidating the main characteristics of AIH, PBC, and PSC, with particular interest for the clinical manifestations, pathogenesis, autoantibodies, and therapeutic options. Immunoglobulin 4(IgG4)-mediated cholangitis is a more recently reported clinical entity belonging to the group of immune-mediated hepatobiliary disorders, and will therefore also be briefly discussed in the present chapter.