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Autoimmune Hepatitis: 2019 Update

机译：自身免疫性肝炎：2019年更新

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Autoimmune hepatitis (AIH) is a chronic inflammatory liver disease, characterized by the elevation of aminotransferases, presence of anti-nuclear antibody or anti-smooth muscle antibody, elevated immunoglobulin G (IgG), and interface hepatitis/plasma-lymphocytic inflammation based on histology. Recent epidemiological studies have indicated an increasing trend in the prevalence of AIH worldwide, especially in male patients; this trend may suggest the alteration of environmental triggers of disease onset over time. As no disease-specific biomarker or histological finding is currently available, AIH requires a clinical diagnosis, and a validated diagnostic scoring system with acceptable specificity and sensitivity has been proposed. Regarding treatment, corticosteroids and azathioprine are recommended, and in those who exhibit an incomplete response or those who are intolerant to these drugs, second-line therapy, such as mycophenolate mofetil, is considered. Overall, the long-term outcome is excellent in patients with complete biochemical responses, while life-long maintenance treatment may be required since the cessation of immunosuppressive agents frequently leads to the relapse of the disease. Acute-onset AIH does occur, and the diagnosis is very challenging due to the lack of serum autoantibodies or elevated IgG. The unmet needs include earlier diagnosis, intervention with disseminated clinical practice guidelines, and recognition and improvement of patients’ health-related quality of life with the development of novel corticosteroid-free treatment regimens.
机译：自身免疫性肝炎（AIH）是一种慢性炎症性肝病，其特征在于氨基转移酶的升高，抗核抗体或抗滑肌抗体，升高的免疫球蛋白G（IgG），以及基于组织学的界面肝炎/血浆淋巴细胞炎症。最近的流行病学研究表明，全球AIH普遍存在的趋势越来越大，特别是在男性患者中;这种趋势可能表明随着时间的推移，疾病发病的环境触发的改变。由于目前没有疾病特异性的生物标志物或组织学发现，AIH需要临床诊断，并提出了具有可接受的特异性和敏感性的验证的诊断评分系统。关于治疗，推荐皮质类固醇和偶氮嘌呤，并且在表现出不完全反应的人或那些与这些药物不耐受的人，考虑了第二线治疗，例如霉酚酸酯MoFetil。总的来说，长期结果是完全生化反应的患者中，而可能需要终身维护治疗，因为免疫抑制剂的停止经常导致疾病的复发。发生急性发作AIH，由于缺乏血清自身抗体或升高的IgG，诊断非常具有挑战性。未满足的需求包括早期的诊断，干预与传播临床实践指导方针，以及患者与患者的健康有关的生活质量的诊断和改善，随着新的皮质类固醇治疗方案的发展。

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