Translational Autoimmunity: Chapter 15 - Autoimmunity of the liver

Elsevier, Translational Autoimmunity, Autoimmune Diseases in Different Organs, Volume 4 in Translational Immunology, 2022, Pages 309-331
Angelo Armandi, Giovanni Clemente Actis, and Davide Giuseppe Ribaldone

Autoimmune hepatitis (AIH) is a chronic inflammatory disease, in which a loss of tolerance against liver antigens is considered the main pathogenic mechanism. AIH is probably triggered by environmental agents (medications, toxins, other immune-mediated disorders) that act upon genetically susceptible individuals. The global burden of AIH is increasing worldwide, potentially due to multiple environmental factors that impact the genome structure. A loss of immune tolerance and exposure of cryptic antigens from damaged hepatocytes are believed to be the strongest driver for the onset and maintenance of an immune-mediated chronic inflammatory injury. The whole clinical spectrum is characterized by unspecific elements, including serum autoantibodies and diverse disease features at liver histology. Considering its increasing prevalence, many efforts are being taken to treat patients who do not adequately respond to standard immunosuppressive treatments as well as to find novel noninvasive biomarkers that can reliably substitute liver histology in assessing liver fibrosis and in predicting hard long-term outcomes.