National Institute for Health Research (NIHR) Birmingham Biomedical Research Centre (BRC), Centre for Liver and Gastroenterology Research, University of Birmingham, UK; Liver Unit, University Hospitals Birmingham, Birmingham, UK; Institute of Immunology and Immunotherapy, University of Birmingham, UK.
National Institute for Health Research (NIHR) Birmingham Biomedical Research Centre (BRC), Centre for Liver and Gastroenterology Research, University of Birmingham, UK; Liver Unit, University Hospitals Birmingham, Birmingham, UK; Institute of Immunology and Immunotherapy, University of Birmingham, UK; Dept. of Cellular Pathology, University Hospitals Birmingham, UK.
J Hepatol. 2019 Apr;70(4):773-784. doi: 10.1016/j.jhep.2018.11.006. Epub 2018 Nov 20.
Autoimmune hepatitis is a corticosteroid-responsive liver disease arising consequent to immunogenetic and environmental risk factors. The clinical course reflects relapsing and remitting, hepatocyte targeted immunologic damage, which is countered by reparative responses to cell injury. Appropriate and timely immunosuppressive therapy drives the disease into remission, albeit with inevitable side effects. Many challenges faced in the clinic reflect practice that must capture a heterogeneous disease presentation, course, and treatment response, as well as treatment tolerability. In this Grand Round we appraise the evidence supporting current treatment approaches, address the impact of autoimmune liver disease 'crossover or overlap' presentations, explore important clinical correlates to immune-serological classifiers, and discuss the factors influencing choice of alternative therapy in difficult-to-treat situations.
自身免疫性肝炎是一种由免疫遗传和环境风险因素引起的皮质类固醇反应性肝脏疾病。临床病程反映了反复发作和缓解，针对肝细胞的免疫损伤，而细胞损伤的修复反应则与之对抗。适当和及时的免疫抑制治疗可使疾病缓解，但不可避免地会产生副作用。临床上面临的许多挑战反映了必须捕捉异质性疾病表现、病程和治疗反应以及治疗耐受性的实践。在本次大查房中，我们评估了支持当前治疗方法的证据，探讨了自身免疫性肝病“交叉或重叠”表现的影响，探讨了免疫血清学分类器的重要临床相关性，并讨论了影响在难治性情况下选择替代治疗的因素。
