Review
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World J Gastroenterol. Jul 28, 2013; 19(28): 4455-4463
Published online Jul 28, 2013. doi: 10.3748/wjg.v19.i28.4455
Autoimmune hepatitis in childhood: The role of genetic and immune factors
Priscila Menezes Ferri Liu, Débora Marques de Miranda, Eleonora Druve Tavares Fagundes, Alexandre Rodrigues Ferreira, Ana Cristina Simões e Silva
Priscila Menezes Ferri Liu, Débora Marques de Miranda, Eleonora Druve Tavares Fagundes, Alexandre Rodrigues Ferreira, Ana Cristina Simões e Silva, Department of Pediatrics, Interdisciplinary Laboratory of Medical Investigation, Federal University of Minas Gerais, Belo Horizonte, Minas Gerais 30130-100, Brazil
Débora Marques de Miranda, Ana Cristina Simões e Silva, National Institute of Science and Technology of Molecular Medicine, (INCT-MM) CNPq-FAPEMIG, Federal University of Minas Gerais, Belo Horizonte, Minas Gerais 30130-100, Brazil
Author contributions: Miranda DM, Fagundes EDT and Ferreira AR designed the research; Ferri Liu PM performed the research; Ferri Liu PM, Miranda DM, Ferreira AR and Simoes e Silva AC wrote the paper; all authors reviewed final version.
Supported by CAPES, INCT-MM (FAPEMIG: CBB-APQ-00075-09/CNPq 573646/2008-2)
Correspondence to: Ana Cristina Simões e Silva, MD, PhD, Department of Pediatrics, Interdisciplinary Laboratory of Medical Investigations, Federal University of Minas Gerais, Avenida Alfredo Balena 190, 2nd floor, Room 281, Belo Horizonte, Minas Gerais 30130-100, Brazil. acssilva@hotmail.com
Telephone: +55-31-34098073 Fax: +55-31-34099772
Received: April 7, 2013
Revised: June 5, 2013
Accepted: June 8, 2013
Published online: July 28, 2013
Processing time: 113 Days and 1.3 Hours
Abstract

Autoimmune hepatitis (AIH) is a rare chronic inflammatory disease of the liver, which affects a group of patients who lost their immunological tolerance to antigens of the liver. It is clinically characterized by hypergammaglobulinemia, elevated liver enzymes, presence of autoantibodies and histological changes. Although being rare in children, it represents a serious cause of chronic hepatic disease that can lead to cirrhosis and hepatic failure. Clinical findings, exclusion of more common liver disorders and the detection of antibodies antinuclear antibodies, smooth muscle antibodies and anti-LKM1 are usually enough for diagnosis on clinical practice. The pathogenic mechanisms that lead to AIH remain obscure, but some research findings suggest the participation of immunologic and genetic factors. It is not yet knew the triggering factor or factors that stimulate inflammatory response. Several mechanisms proposed partially explain the immunologic findings of AIH. The knowledge of immune factors evolved might result in better markers of prognosis and response to treatment. In this review, we aim to evaluate the findings of research about genetic and immune markers and their perspectives of application in clinical practice especially in pediatric population.

Keywords: Autoimmune hepatitis; Genetics; Clinical practice; Immunophenotype

Core tip: In this review article, we reported recent data on autoimmune hepatitis in pediatric patients highlighting the importance of genetic and immune markers. We also discuss the perspectives of the application of these new biomarkers in clinical practice.