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©2014 Baishideng Publishing Group Inc. All rights reserved.
World J Hepatol. Jul 27, 2014; 6(7): 464-476
Published online Jul 27, 2014. doi: 10.4254/wjh.v6.i7.464
Published online Jul 27, 2014. doi: 10.4254/wjh.v6.i7.464
Juvenile autoimmune hepatitis: Spectrum of the disease
Giuseppe Maggiore, Silvia Nastasio, Department of Clinical and Experimental Medicine, Pediatric Gastroenterology, University Hospital of Pisa, 56127 Pisa, Italy
Marco Sciveres, Pediatric Hepatology and Pediatric Liver Transplantation, UPMC Ismett, 90100 Palermo, Italy
Author contributions: All authors equally contributed to this paper.
Correspondence to: Giuseppe Maggiore, MD, Department of Clinical and Experimental Medicine, Pediatric Gastroenterology, University Hospital of Pisa, Via Roma 67, 56127 Pisa, Italy. giuseppe.maggiore@med.unipi.it
Telephone: + 39-50-992639 Fax: + 39-50-993044
Received: November 7, 2013
Revised: May 19, 2014
Accepted: May 29, 2014
Published online: July 27, 2014
Revised: May 19, 2014
Accepted: May 29, 2014
Published online: July 27, 2014
Core Tip
Core tip: Juvenile autoimmune hepatitis is an inflammatory liver disease affecting mainly young girls from infancy to late adolescence, characterized by active liver damage, elevated immunoglobulin G levels, high titers of serum non organ-specific and organ-specific autoantibodies, and interface hepatitis on liver biopsy. Two types are identified according to the autoantibody panel, with differences in the epidemiological distribution, genetic markers and clinical presentation. The most effective therapy for autoimmune hepatitis is pharmacological suppression of the immune response. Treatment should be started as soon as the diagnosis is made to avoid severe liver damage and progression of fibrosis.