Review
Copyright ©The Author(s) 2024. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Hepatol. Nov 27, 2024; 16(11): 1225-1242
Published online Nov 27, 2024. doi: 10.4254/wjh.v16.i11.1225
Autoimmune hepatitis: Towards a personalized treatment
Alejandro Costaguta, Guillermo Costaguta, Fernando Álvarez
Alejandro Costaguta, Department of Hepatology and Liver Transplant Unit, Sanatorio de Niños de Rosario, Rosario 2000, Santa Fe, Argentina
Guillermo Costaguta, Department of Gastroenterology, Hepatology, and Nutrition, CHU Sainte-Justine, Montreal H3T 1C5, Quebec, Canada
Fernando Álvarez, Department of Pediatrics, CHU Sainte-Justine, Montreal H3T 1C5, Quebec, Canada
Co-first authors: Alejandro Costaguta and Guillermo Costaguta.
Author contributions: Costaguta A contributed to the conception of the work, data collection and review, critical revision of the final draft, and approval for publication; Costaguta G contributed to conception of the work, collection of data, draft of the first version, and approval for publication; Álvarez F contributed to data collection, critical review of the final draft, and approval for publication.
Conflict-of-interest statement: The authors declare that they have no conflicts of interest.
Open-Access: This article is an open-access article that was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution NonCommercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: https://creativecommons.org/Licenses/by-nc/4.0/
Corresponding author: Alejandro Costaguta, MD, Chief Physician, Doctor, Staff Physician, Department of Hepatology and Liver Transplant Unit, Sanatorio de Niños de Rosario, Alvear 863, CP 2000, Rosario 2000, Santa Fe, Argentina. alejandro.costaguta@gmail.com
Received: June 25, 2024
Revised: September 2, 2024
Accepted: October 11, 2024
Published online: November 27, 2024
Processing time: 132 Days and 20.2 Hours
Abstract

Autoimmune hepatitis is an uncommon condition that affects both adults and children and is characterized by chronic and recurrent inflammatory activity in the liver. This inflammation is accompanied by elevated IgG and autoantibody levels. Historically, treatment consists of steroids with the addition of azathioprine, which results in remission in approximately 80% of patients. Despite significant advancements in our understanding of the immune system over the past two decades, few modifications have been made to treatment algorithms, which have remained largely unchanged since they were first proposed more than 40 years ago. This review summarized the various treatment options currently available as well as our experiences using them. Although steroids are the standard treatment for induction therapy, other medications may be considered. Cyclosporin A, a calcineurin inhibitor that decreases T cell activation, has proven effective for induction of remission, but its long-term side effects limit its appeal for maintenance. Tacrolimus, a drug belonging to the same family, has been used in patients with refractory diseases with fewer side effects. Sirolimus and everolimus have interesting effects on regulatory T cell populations and may become viable options in the future. Mycophenolate mofetil is not effective for induction but is a valid alternative for patients who are intolerant to azathioprine. B cell-depleting drugs, such as rituximab and belimumab, have been successfully used in refractory cases and are useful in both the short and long term. Other promising treatments include anti-tumor necrosis factors, Janus kinases inhibitors, and chimeric antigen receptor T cell therapy. This growing armamentarium allows us to imagine a more tailored approach to the treatment of autoimmune hepatitis in the near future.

Keywords: Novel therapies; Immunosuppressors; Treatment tailoring; Personalization; Special populations

Core Tip: Autoimmune hepatitis is a serious condition that affects both adults and children and has a poor prognosis if left untreated. Historically, treatment has involved high-dose steroids to induce remission and maintenance therapy with azathioprine, which achieves remission in 80% of cases but requires alternative therapies for the remaining 20%. While there have been advancements in immunosuppressive therapies for other diseases, treatment protocols for autoimmune hepatitis have remained largely unchanged. In this article, we explored alternative treatment options for patients for whom the standard protocol is not suitable.