Clinical significance of autoantibodies in autoimmune hepatitis |
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| Affiliation: | 1. Paediatric Liver Centre and Institute of Liver Studies, King''s College London School of Medicine at King''s College Hospital, Denmark Hill, London SE5 9RS, UK;2. Faculty of Medicine, University of Porto, Porto, Portugal;1. Keck School of Medicine, University of Southern California, Los Angeles, USA;2. Liver/Intestinal Transplant and Hepatology Program, Children''s Hospital of Los Angeles, 4650 Sunset Blvd, #147, Los Angeles, CA 90027, USA;3. Loma Linda University Children''s Hospital, Loma Linda, CA 92354, USA;1. First Department of Internal Medicine, San Matteo Hospital Foundation, University of Pavia, Pavia, Italy;2. Department of Medical and Surgical Sciences, Sant’Orsola-Malpighi, University of Bologna, Bologna, Italy;3. Department of Medicine, Pancreas Center, University of Verona, Verona, Italy;1. Epatocentro Ticino, Lugano, Switzerland;2. Neurocentro della Svizzera Italiana, Ospedale regionale di Lugano, Lugano, Switzerland;3. Fondazione Epatocentro Ticino, Lugano, Switzerland;4. Servizio di Epatologia EOC, Bellinzona, Switzerland;5. LUM Autoimmunity and Allergy AUSL Bologna, Italy;6. Paediatric Liver, GI and Nutrition Centre, MowatLabs, King''s College Hospital, London, UK;7. Institute of Liver Studies, MowatLabs, King''s College Hospital, London, UK;8. DIMEC, University of Bologna, Bologna, Italy;1. Division of Gastroenterology, Hepatology and Nutrition, Golisano Childrens Hospital at Strong, University of Rochester Medical Center, 601 Elmwood Avenue, Box 667 Rochester, New York 14642 USA;2. Institute of Liver Studies, Mowat Labs, King''s College Hospital, London, United Kingdom |
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| Abstract: | The accurate diagnosis and classification of autoimmune hepatitis (AIH) rely upon the detection of characteristic autoantibodies. Positivity for anti-nuclear (ANA) and/or anti-smooth muscle (SMA) autoantibodies defines AIH type 1 (AIH-1), whereas anti-liver kidney microsomal type 1 (anti-LKM1) and/or anti-liver cytosol type 1 (anti-LC1) define AIH type 2 (AIH-2). ANA and SMA, and less commonly anti-LKM1, have also been detected in de-novo autoimmune hepatitis developing after liver transplantation, a condition that may affect patients transplanted for non-autoimmune liver disease. The diagnostic autoantibodies associated with AIH-1 are also detected in the paediatric AIH/sclerosing cholangitis overlap syndrome, referred to as autoimmune sclerosing cholangitis (ASC). ASC, like adult primary sclerosing cholangitis, is often associated with atypical perinuclear anti-neutrophil cytoplasmic autoantibodies (p-ANCA), although p-ANCA are also detected in other autoimmune liver diseases. These associations highlight the necessity for simple and prompt diagnostic autoantibody testing, and the requirement for the accurate interpretation of the results of the tests in the clinical context. Fine-mapping of antigenic autoantibody targets has facilitated the development of rapid molecular assays that have the potential to revolutionise the field if properly standardised and when used in combination with classical immunofluorescence. Despite their diagnostic significance, the pathogenic role of the various autoantibodies and the mechanisms by which they can potentially inflict damage onto the liver cell remain a topic for further research. |
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| Keywords: | Autoimmune hepatitis Anti-nuclear antibody Anti-smooth muscle antibody Anti-liver kidney microsomal antibody Anti-liver cytosol antibody Anti-soluble liver antigen |
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