Diagnostic criteria of autoimmune hepatitis |
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| Institution: | 1. Paediatric Liver, GI & Nutrition Centre and Institute of Liver Studies, King''s College London School of Medicine at King''s College Hospital, London, UK;2. Faculty of Medicine, University of Porto, Porto, Portugal;1. Allergologia e Immunologia Clinica, A.O. S. Maria degli Angeli, Pordenone, Italy;2. Division of Transplantation Immunology and Mucosal Biology, King''s College London School of Medicine at King''s College Hospital, London, UK;3. GA Generic Assays GmbH, Dahlewitz, Germany;4. Faculty of Sciences, Brandenburg University of Technology Cottbus-Senftenberg, Senftenberg, Germany;5. Department or Rheumatology, School of Health Sciences, University of Thessaly, Larissa, Greece |
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| Abstract: | Autoimmune hepatitis (AIH) is a chronic immune-mediated liver disorder characterised by female preponderance, elevated transaminase and immunoglobulin G levels, seropositivity for autoantibodies and interface hepatitis. Presentation is highly variable, therefore AIH should be considered during the diagnostic workup of any increase in liver enzyme levels. A set of inclusion and exclusion criteria for the diagnosis of AIH have been established by the International Autoimmune Hepatitis Group (IAIHG). There are two main types of AIH: type 1, positive for anti-nuclear (ANA) and/or anti-smooth muscle antibodies (SMAs) and type 2, defined by the presence of anti-liver kidney microsomal antibody type 1 (LKM-1) and/or anti-liver cytosol type 1 (LC-1) autoantibodies. The central role of autoantibodies in the diagnosis of AIH has led the IAIHG to produce a consensus statement detailing appropriate and effective methods for their detection. Autoantibodies should be tested by indirect immunofluorescence at an initial dilution of 1/40 in adults and 1/10 in children on a freshly prepared rodent substrate that includes kidney, liver and stomach sections to allow for the simultaneous detection of all reactivities relevant to AIH. Anti-LKM-1 is often confused with anti-mitochondrial antibody (AMA) if rodent kidney is used as the sole immunofluorescence substrate. The identification of the molecular targets of anti-LKM-1 and AMA has led to the establishment of immuno-assays based on the use of the recombinant or purified autoantigens. Perinuclear anti-nuclear neutrophil antibody (p-ANNA) is an additional marker of AIH-1; anti soluble liver antigen (SLA) antibodies are specific for autoimmune liver disease, can be present in AIH-1 and AIH-2 and are associated with a more severe clinical course. Anti-SLA are detectable by ELISA or radio-immuno-assays, but not by immunofluorescence. AIH is exquisitely responsive to immunosuppressive treatment, which should be instituted promptly to prevent rapid deterioration and promote remission and long-term survival. |
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