REVIEW  AUTOIMMUNE LIVER DISEASES 

Minerva Gastroenterology 2023 March;69(1):61-83

DOI: 10.23736/S2724-5895.20.02805-6

Copyright © 2020 EDIZIONI MINERVA MEDICA

language: English

Open challenges in the management of autoimmune hepatitis

Alessio GERUSSI ^{1, 2, 3} ✉, Neil HALLIDAY ⁴, Marco CARBONE ^{1, 2}, Pietro INVERNIZZI ^{1, 2}, Douglas THORBURN ⁴

¹ Division of Gastroenterology, Department of Medicine and Surgery, Center for Autoimmune Liver Diseases, University of Milano-Bicocca, Monza, Monza-Brianza, Italy; ² European Reference Network on Hepatological Diseases (ERN RARE-LIVER), San Gerardo Hospital, Monza, Monza-Brianza, Italy; ³ Ancient DNA Lab Dan David Center for Human Evolution and Biohistory Research, Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel; ⁴ Institute for Liver and Digestive Health, University College London, London, UK; ⁵ European Reference Network on Hepatological Diseases (ERN RARE-LIVER), The Sheila Sherlock Liver Centre, Royal Free Hospital, London, UK

Autoimmune hepatitis (AIH) is a rare autoimmune disease of the liver with many open questions as regards its etiopathogenesis, natural history and clinical management. The classical picture of AIH is chronic hepatitis with fluctuating elevation of serum transaminases and Immunoglobulin G levels, the presence of circulating autoantibodies and typical histological features. However, atypical presentations do occur and are not well captured by current diagnostic scores, with important consequences in terms of missed diagnoses and delayed treatments. AIH is treated with corticosteroids and immunosuppressive drugs but up to 40% of patients do not achieve full biochemical response and are at risk of progressing to cirrhosis and liver failure. Moreover, standard therapies are associated by significant side-effects which may impair the quality of life of patients living with AIH. However, advances in the understanding of the underlying immunology of AIH is raising the prospect of novel therapies and optimization of existing therapeutic approaches to reduce side-effect burdens and potentially restore immunological tolerance. In this review we outlined the clinical characteristics, etiopathogenesis and management of AIH and current challenges in the diagnosis and management of AIH and provided evidence underlying the evolution of diagnostic and clinical management protocols.

KEY WORDS: Autoimmunity; Liver transplantation; Liver cirrhosis; Liver diseases