title: Autoimmune Hepatitis: Clinical Review with Insights into the Purinergic Mechanism of Disease.
abstract: Autoimmune hepatitis (AIH) is an important disorder that predominantly results in inflammatory liver disease in genetically predisposed women. The clinicopathological picture is characterized by symptoms associated with both systemic inflammation and hepatic dysfunction, and with increased serum aminotransferases, elevated IgG, autoantibodies, and interface hepatitis on liver biopsy. AIH usually results in liver injury as a consequence of chronic hepatitis and cirrhosis. However, rarely, patients may present with fulminant liver failure. Early diagnosis is important in all instances because the disease can be highly responsive to immunosuppressive therapeutic options. Left untreated, the disease is associated with high morbidity and mortality. Here we provide an overview of the current state of knowledge on AIH and summarize the treatment options for this serious condition in adults. We also discuss the pathogenesis of the disease as a possible consequence of autoimmunity and the breakdown of hepatic tolerance. We focus on regulatory T cell impairments as a consequence of changes in CD39 ectonucleotidase expression and altered purinergic signaling. Further understanding of hepatic tolerance may aid in the development of specific and well-tolerated therapies for AIH.
authors.affiliation: Department of Medicine, University of Connecticut, Farmington, CT, USA ; These authors contributed equally to this work.
authors.forename: Nikhil
authors.initials: N
authors.lastname: Kapila
authors.affiliation: Gastroenterology Division and Department of Medicine, Beth Israel Deaconess Medical Center and Harvard Medical School, Boston, MA, USA ; These authors contributed equally to this work.
authors.forename: Jennifer T
authors.initials: JT
authors.lastname: Higa
authors.affiliation: Gastroenterology Division and Department of Medicine, Beth Israel Deaconess Medical Center and Harvard Medical School, Boston, MA, USA ; Institute of Liver Studies, King's College London School of Medicine at King's College Hospital, Denmark Hill, London, UK.
authors.forename: Maria Serena
authors.initials: MS
authors.lastname: Longhi
authors.affiliation: Gastroenterology Division and Department of Medicine, Beth Israel Deaconess Medical Center and Harvard Medical School, Boston, MA, USA.
authors.forename: Simon C
authors.initials: SC
authors.lastname: Robson
keywords: Autoimmune hepatitis; CD39; Immunology; Pathogenesis; Purinergic; Therapeutic overview