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World J Hepatol. Jun 27, 2014; 6(6): 410-418
Published online Jun 27, 2014. doi: 10.4254/wjh.v6.i6.410
Management of autoimmune hepatitis: Focus on pharmacologic treatments beyond corticosteroids
Marta Casal Moura, Rodrigo Liberal, Hélder Cardoso, Ana Maria Horta e Vale, Guilherme Macedo
Marta Casal Moura, Serviço de Medicina Interna, Centro Hospitalar de São João, E.P.E., Porto 4200-319, Portugal
Marta Casal Moura, Rodrigo Liberal, Hélder Cardoso, Ana Maria Horta e Vale, Guilherme Macedo, Faculdade de Medicina da Universidade do Porto, Hernâni Monteiro, Porto 4200-319, Portugal
Rodrigo Liberal, Paediatric Liver Centre and Institute of Liver Studies, King’s College London School of Medicine at King’s College Hospital, London SE5 9RS, United Kingdom
Hélder Cardoso, Ana Maria Horta e Vale, Guilherme Macedo, Serviço de Gastrenterologia, Centro Hospitalar de São João, E.P.E., Porto 4202-451, Portugal
Author contributions: Casal Moura M and Liberal R design and performed the research; Casal Moura M and Liberal R wrote the paper; Cardoso H, Horta e Vale AM and Macedo G reviewed the paper.
Correspondence to: Marta Casal Moura, MD, Alameda Professor, Faculdade de Medicina da Universidade do Porto, Hernâni Monteiro, Porto 4200-319, Portugal. martacasalmoura@gmail.com
Telephone: +3-51-225512100 Fax: +3-51-22502576
Received: November 5, 2013
Revised: April 19, 2014
Accepted: May 8, 2014
Published online: June 27, 2014
Processing time: 241 Days and 10.4 Hours
Abstract

In autoimmune hepatitis, patients who are intolerant or with toxicity experience, non-responders, relapsers or refractory are challenging. Non-standard drugs are being tried to preemptively avoid corticosteroid-related side effects. Prognosis and quality of life of life rely on treatment optimization. Recently, emergence of powerful immunosuppressive agents, mainly from liver transplantation, challenged the supremacy of the corticosteroid regime and promise greater immunosuppression than conventional medications, offer site-specific actions and satisfactory patient tolerance. Successes in experimental models of related diseases have primed these molecular interventions. We performed a literature review on alternative treatments. Azatioprine intolerance is the principal indication for mycophenolate use but it can be used as a front-line therapy. Cyclosporine A and tacrolimus have been tested for non-responders or relapsers. Rituximab may be used as salvage therapy. Anti-tumor necrosis factor-alpha agents may be used for incomplete responses or non-responders. Methotrexate is possibly an alternative for induction of remission and maintenance in refractory patients. Cyclophosphamide has been included in the induction regimen with corticosteroids. Ursodeoxycholic acid action is mainly immunomodulatory. Non-standard treatments are coming slowly to the attention, but its use should be cautious performed by experienced centers.

Keywords: Autoimmune hepatitis; Pharmacologic non-standard treatment; Immunosuppression; Azathioprine intolerance; Difficult-to-treat patients; Salvage therapy

Core tip: With our review we pretend to describe the non-standard pharmacologic treatments available for autoimmune hepatitis, the indications for its use and the main applications. Also, we pretend to enhance that those alternatives are only available guided by the experience in liver transplant patients and should be only used by experienced centers. The difficult-to-treat patients lead to the application of those therapies mainly as salvage treatments.