Autoimmune hepatitis (AIH) is an organ specific autoimmune condition which can manifest at any age of life. The heterogeneous nature of this condition means that great variation can be seen in severity, progression of disease and response to treatment within this patient group. Since the 1980s prednisolone and azathioprine have been used for induction and remission of the disease and remain the mainstay of treatment. Other immunosuppressive agents have been employed in difficult to treat cases. While there is less published data regarding these agents compared with the conventional treatments of steroid and azathioprine, there is mounting evidence to support the use of mycophenolate mofetil as a second-line agent. The calcineurin inhibitors, though less studied, additionally show promise. More data is needed on the use of biological agents in refractory disease. This review focuses on our centre’s approach to treatment of AIH in the context of a contemporary review of the literature.
Citations
Citations to this article as recorded by
Assessing the associations between immunosuppressant use and COVID-19 severity in patients with autoimmune hepatitis Krish Rai, Karla J Lindquist, John Kornak, Jin Ge World Journal of Hepatology.2026;[Epub] CrossRef
Global, regional, and national burden of nonalcoholic fatty liver disease among adults aged ≥ 45 years: A comprehensive analysis of epidemiological trends and projections to 2035 Qian Wang, Jieru Guo, Shuang Liu, Xuebin Cao, Zhirong Guo, Long Rui, Liu Zheng, Chenyang Wang, Tiejun Zhang PLOS One.2026; 21(3): e0342697. CrossRef
Comment on “Paediatric-onset autoimmune liver disease: Insights from a monocentric experience” Qateen Zahra, Javeria Zulfiqar Cheema, Muhammad Rafay Shahzad Cheema Digestive and Liver Disease.2025; 57(3): 797. CrossRef
The safety and efficacy of rituximab in autoimmune hepatitis: a systematic review and quality assessment Mohammad Amin Habibi, Fatemeh Aghayee, Mohammad Sina Mirjani, Mohammad Reza Karimifar, Mohammad Reza Ahmadi, Seyed Mohammad Eazi, Poriya Minaee, Mohammad Reza Pashaei, Ahmad Hormati, Mohammad Mahdi Akbari Aleagha, Sajjad Ahmadpour European Journal of Gastroenterology & Hepatology.2025; 37(10): 1097. CrossRef
Autoimmune hepatitis presenting with concomitant chronic pancreatitis Dhruv Patel, Ahmed Salem, Brooke Kania, William Lewis, Anas Mahmoud, Mina Alkomos Radiology Case Reports.2023; 18(9): 2871. CrossRef
Pharmacokinetic/Pharmacodynamic Assessment of Selective Phosphodiesterase Inhibitors in a Mouse Model of Autoimmune Hepatitis Artur Świerczek, Bartosz Pomierny, Elżbieta Wyska, William J. Jusko The Journal of Pharmacology and Experimental Therapeutics.2022; 381(2): 151. CrossRef
Historical aspects and current understanding of autoimmune hepatitis. When is liver transplantation indicated? (Review) I. M. Iljinsky, O. M. Tsirulnikova Russian Journal of Transplantology and Artificial Organs.2022; 24(2): 39. CrossRef
Myeloid-derived suppressor cells as cellular immunotherapy in transplantation and autoimmune diseases Jilu Zhang, Alan Hodges, Shu-Hsia Chen, Ping-Ying Pan Cellular Immunology.2021; 362: 104300. CrossRef
PK/PD Modeling of the PDE7 Inhibitor—GRMS-55 in a Mouse Model of Autoimmune Hepatitis Artur Świerczek, Hanna Plutecka, Marietta Ślusarczyk, Grażyna Chłoń-Rzepa, Elżbieta Wyska Pharmaceutics.2021; 13(5): 597. CrossRef
Rescue of autoimmune hepatitis by soluble MHC class II molecules in an altered concanavalin A‐induced experimental model Katerina Bakela, Maria Georgia Dimitraki, Evangelia Skoufa, Irene Athanassakis Animal Models and Experimental Medicine.2020; 3(3): 264. CrossRef
Autoimmune Hepatitis and Stellate Cells: An Insight into the Role of Autophagy Shahram Golbabapour, Kamran Bagheri-Lankarani, Saeid Ghavami, Bita Geramizadeh Current Medicinal Chemistry.2020; 27(35): 6073. CrossRef
An intensive medical care network led to successful living-donor liver transplantation in late-onset hepatic failure with disseminated Staphylococcus aureus infection Rie Kure, Natsumi Uehara, Kazuaki Inoue, Tomomi Kogiso, Kazuhisa Kodama, Makiko Taniai, Katsutoshi Tokushige, Masayuki Nakano, Hiroto Egawa, Masakazu Yamamoto Clinical Journal of Gastroenterology.2019; 12(2): 112. CrossRef
Inside autoimmune liver disease Richard L. Pullen, Patricia Francis-Johnson Nursing Made Incredibly Easy!.2019; 17(1): 40. CrossRef
Methylprednisolone Decreases Mitochondria-Mediated Apoptosis and Autophagy Dysfunction in Hepatocytes of Experimental Autoimmune Hepatitis Model via the Akt/mTOR Signaling Xiaoli Fan, Ruoting Men, Haoran Wang, Mengyi Shen, Tingting Wang, Tinghong Ye, Xuefeng Luo, Li Yang Frontiers in Pharmacology.2019;[Epub] CrossRef
Efficacy of rituximab in difficult-to-manage autoimmune hepatitis: Results from the International Autoimmune Hepatitis Group Nwe Ni Than, James Hodson, Daniel Schmidt-Martin, Richard Taubert, Rebecca E. Wawman, Meemee Botter, Nishant Gautam, Kilian Bock, Rebecca Jones, Gautham D Appanna, Andrew Godkin, Aldo J. Montano-Loza, Frank Lammert, Christoph Schramm, Michael P. Manns, Ma JHEP Reports.2019; 1(6): 437. CrossRef
Thrombotic thrombocytopenic purpura and defective apoptosis due to CASP8/10 mutations: the role of mycophenolate mofetil F. Fioredda, E. Cappelli, A. Mariani, A. Beccaria, E. Palmisani, A. Grossi, I. Ceccherini, R. Venè, C. Micalizzi, M. Calvillo, F. Pierri, I. Mancini, F. Peyvandi, F. Corsolini, C. Dufour, M. Miano Blood Advances.2019; 3(21): 3432. CrossRef
Transient elastography to assess liver stiffness in patients with inflammatory bowel disease Berrie Meijer, Charlotte K. van Everdingen, Dewkoemar Ramsoekh, Catherine Stedman, Christopher M.A. Frampton, Chris J.J. Mulder, Gerd Bouma, Nanne K.H. de Boer, Richard B. Gearry Digestive and Liver Disease.2018; 50(1): 48. CrossRef
Rheumatic Manifestations in Autoimmune Liver Disease Carlo Selmi, Elena Generali, Merrill Eric Gershwin Rheumatic Disease Clinics of North America.2018; 44(1): 65. CrossRef
Current and future perspectives in autoimmune hepatitis Eleni Theocharidou, Michael A Heneghan British Journal of Hospital Medicine.2018; 79(3): 151. CrossRef
A RARE CASE OF CROHN DISEASE COMPLICATED WITH STEROID MONOTHERAPY-RELATED RETROPHARYNGEAL ABSCESS AND INITIALLY MISINTERPRETED PYODERMA GANGRENOSUM DEVELOPMENT Michael Doulberis, Jörg Dähn, Jannis Kountouras, Volker Maier, Arthur Helbling, Patrick Dubach Gastroenterology Nursing.2018; 41(4): 347. CrossRef
Comparative efficacy and tolerability of treatments for adult autoimmune hepatitis: A systematic review and network meta‑analysis Feng‑Bin Lu, En‑De Hu Hu, Lan‑Man Xu, Yi‑Bing Hu, Lu Chen, Jin‑Lu Wu, Hui Li, Da‑Zhi Chen, Yong‑Ping Chen Experimental and Therapeutic Medicine.2018;[Epub] CrossRef
Undiagnosed autoimmune hepatitis causing prolonged mechanical ventilation DavidW Mattingley, ThomasJ Papadimos International Journal of Critical Illness and Injury Science.2018; 8(3): 179. CrossRef
Autoimmune hepatitis (AIH) is an immune-mediated chronic liver disease characterized by hepatocellular inflammation, necrosis, and fibrosis, which can progress to cirrhosis and fulminant hepatic failure. The standard treatment for AIH includes corticosteroids alone or in combination with azathioprine. Although most patients achieve remission using the standard regimen, some patients do not respond due to either drug intolerance or refractory disease; in such cases alternative immunosuppressive agents should be explored. The second-line therapies are cyclophilin inhibitors such as cyclosporine A or tacrolimus, and nowadays mycophenolate mofetil (MMF) is widely used if azathioprine-based therapies are not tolerated. Although these are recommended as an alternative to the first-line regimen, there is insufficient evidence for the efficacy of second-line therapies, with the evidence based mainly on expert opinion. Therefore, we report an AIH patient receiving the standard regimen in whom remission did not occur due to side effects to azathioprine, but was successfully treated with MMF in combination with corticosteroids as an alternative to the standard regimen.
Citations
Citations to this article as recorded by
Revealing the pathogenesis of autoimmune hepatitis and research progress in drug discovery from hepatic immune cells and intercellular communication signaling mechanisms Jie Yang, Hongying Zhou, Xiao Ma, Xuelin Zhou, Shizhang Wei, Yanling Zhao, Chunyu Li Frontiers in Immunology.2026;[Epub] CrossRef
Cytopenias in Autoimmune Liver Diseases—A Review Mohammed Abdulrasak, Ali M. Someili, Mostafa Mohrag Journal of Clinical Medicine.2025; 14(5): 1732. CrossRef
Inflammatory bowel disease and risk of autoimmune hepatitis: A univariable and multivariable Mendelian randomization study Gang Chi, Jinhong Pei, Xueqing Li, Alessandro Granito PLOS ONE.2024; 19(6): e0305220. CrossRef
Mycophenolate mofetil as a treatment for presumed idiopathic chronic hepatitis in dogs: Six cases (2010–2022) Michelle K. Beehler, Shawn A. Kearns, Zachary J. Crouse Veterinary Medicine and Science.2023; 9(6): 2527. CrossRef
Alcohol-associated liver disease: A review on its pathophysiology, diagnosis and drug therapy Vetriselvan Subramaniyan, Srikumar Chakravarthi, Ravindran Jegasothy, Wu Yuan Seng, Neeraj Kumar Fuloria, Shivkanya Fuloria, Iswar Hazarika, Anju Das Toxicology Reports.2021; 8: 376. CrossRef
Human Cytomegalovirus Upregulates Expression of HCLS1 Resulting in Increased Cell Motility and Transendothelial Migration during Latency Yusuf Aslam, James Williamson, Veronika Romashova, Elizabeth Elder, Benjamin Krishna, Mark Wills, Paul Lehner, John Sinclair, Emma Poole iScience.2019; 20: 60. CrossRef
Efficacy and Safety of Mycophenolate Mofetil in Patients With Autoimmune Hepatitis and Suboptimal Outcomes After Standard Therapy Stuart K. Roberts, Ricky Lim, Simone Strasser, Amanda Nicoll, Alessia Gazzola, Joanne Mitchell, Way Siow, Tiffany Khoo, Zaki Hamarneh, Martin Weltman, Paul Gow, Natasha Janko, Edmund Tse, Gauri Mishra, En-Hsiang Cheng, Miriam Levy, Wendy Cheng, Siddharth Clinical Gastroenterology and Hepatology.2018; 16(2): 268. CrossRef
Alternative treatment regimens in autoimmune hepatitis: how justified is their choice? M. V. Matsievich, A. O. Bueverov, M. Yu. Petrachenkova Almanac of Clinical Medicine.2018; 46(5): 504. CrossRef
Comparative efficacy and tolerability of treatments for adult autoimmune hepatitis: A systematic review and network meta‑analysis Feng‑Bin Lu, En‑De Hu Hu, Lan‑Man Xu, Yi‑Bing Hu, Lu Chen, Jin‑Lu Wu, Hui Li, Da‑Zhi Chen, Yong‑Ping Chen Experimental and Therapeutic Medicine.2018;[Epub] CrossRef
Diagnosis and Treatment of Autoimmune Liver Diseases in a Tertiary Referral Center in Cuba Marlen Ivón Castellanos Fernández, Deyanira la Rosa Hernández, Diego Enrique Cabrera Eugenio, Wilson Palanca, Zaily Dorta Guridi, Licet González Fabián Current Therapeutic Research.2017; 85: 8. CrossRef
Autoimmune hepatitis: Standard treatment and systematic review of alternative treatments Benedetta Terziroli Beretta-Piccoli, Giorgina Mieli-Vergani, Diego Vergani World Journal of Gastroenterology.2017; 23(33): 6030. CrossRef
First
Prev
