Cutting edge issues in autoimmune hepatitis |
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| Affiliation: | 1. Institute of Liver Studies, King’s College Hospital, London, UK;2. Department of Medicine, Dartmouth College, Hanover, NH, USA;3. Department of Medicine, University of Vermont, Burlington, VT, USA;4. Departments of Medicine and Surgery, Baylor College of Medicine, Baylor-St Luke’s Medical Center, Houston, TX, USA;5. Hannover Medical School, Hannover, Germany;6. Paediatric Liver, GI & Nutrition Centre, King’s College Hospital, London, UK;1. Department of Medicine, University Medical Center Hamburg-Eppendorf, Hamburg, Germany;3. Centre for Liver Research, University of Birmingham Liver Unit, Birmingham, United Kingdom;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;1. Division of Gastroenterology and Hepatology, Mayo Clinic College of Medicine, 200 First Street Southwest, Rochester, MN 55905, USA;2. Department of Laboratory Medicine and Pathology, Mayo Clinic College of Medicine, 200 First Street Southwest, Rochester, MN 55905, USA;1. State Key Laboratory of Oncogenes and Related Genes, Key Laboratory of Gastroenterology & Hepatology, Ministry of Health, Division of Gastroenterology and Hepatology, Renji Hospital, School of Medicine, Shanghai Jiao Tong University, Shanghai Cancer Institute, Shanghai Institute of Digestive Disease, 145 Shandong Road, Shanghai 200001, China;2. Department of Medicine, Dartmouth College, Hanover, NH, USA;3. Division of Rheumatology, Allergy, and Clinical Immunology, University of California at Davis, Davis, CA, USA;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 |
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| Abstract: | Autoimmune hepatitis (AIH) is a severe liver disease affecting all age groups worldwide. Novel basic and clinical aspects of AIH, addressed at a Monothematic Conference in London in September 2015, are highlighted in this review. The diagnosis of AIH relies upon detection of characteristic autoantibodies, hypergammaglobulinemia, and interface hepatitis on liver histology. The International Autoimmune Hepatitis Group (IAIHG) has devised diagnostic scoring systems to help in comparative studies and clinical practice. AIH arises in a genetically predisposed host, when yet unknown triggers – such an encounter with a pathogen – lead to a T cell-mediated immune response targeting liver autoantigens. This immune response is inadequately controlled because regulatory mechanisms are impaired. The mainstay of treatment for AIH is immunosuppression, which should be instituted as soon as the diagnosis is made. Standard treatment regimens include relatively high doses of predniso(lo)ne, which are tapered gradually as azathioprine is introduced. Recent guidelines have described newer treatment regimens and have tightened the goal of therapy to complete normalization of biochemical, serological and histological parameters. Mycophenolate mofetil, calcineurin inhibitors, mTOR inhibitors and biological agents are potential salvage therapies, but should be reserved for selected non-responsive patients and administered only in experienced centers. Liver transplantation is a life-saving option for those patients who progress to end-stage liver disease. Further dissection of cellular and molecular pathways involved in AIH pathogenesis is likely to lead to the discovery of novel, tailored and better tolerated therapies. |
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| Keywords: | Autoimmune hepatitis Pathogenesis Immunosuppression International Autoimmune Hepatitis Group |
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