第24届亚太地区肝脏研究协会年会（APASL 2014）于2014年3月12～15日在澳大利亚布里斯班召开。会上来自中国上海交通大学医学院附属仁济医院的马雄医生介绍了亚洲自身免疫性肝炎的诊断和治疗现状。
马雄医生
自身免疫性肝炎（AIH）是因肝细胞异常自身免疫反应而导致的肝脏慢性炎症性疾病。自身免疫性肝炎的发病率为每年0.1-1.9/100,000, 在所有慢性肝病患者中，有11-23%的患者存在这种情况。
AIH的病因目前还不清楚，但许多环境因素、遗传因素和病理生理学因素被认为与其发病有关。AIH的发病机理牵扯到易感人群对肝脏自身抗原的耐受缺失。现已明确，AIH存在靶向作用于肝细胞的自身免疫反应，这和其他自身免疫性疾病，如I型糖尿病、Graves病和增殖性肾小球肾炎有高度相关性。
AIH的诊断依赖于组织学异常、临床特征和生化检查结果，包括血浆球蛋白水平异常、存在一种或多种特征性自身抗体如ANA，SMA和抗SLA。界面性肝炎渗透物中含有大量浆细胞是AIH的特征性组织学特点。简化诊断标准对于诊断亚洲患者AIH有高灵敏性和特异性。原修订版诊断标准有补充作用，避免不典型AIH患者的阴性误诊。
尽管缺乏流行病数据，在中国AIH大量确诊，被认为是非病毒性慢性肝炎的主要病因。越来越多的肝病中心常规性报告肝活检和血浆自身抗体检测结果。因为肝脏病专家和病理医生对AIH组织学特征的警觉和注意，我们医疗中心越来越多的既往认为是原因不明慢性肝炎病例的患者得以确诊AIH。
幸运的是，AIH依靠目前治疗方法能够得到有效控制。免疫抑制治疗可以减弱肝脏炎症，逆转肝纤维化过程，最终改善患者预后，提高生活质量。
AIH对免疫抑制治疗有良好的反应，尽管晚期或合并严重疾病的患者不太可能得到缓解。肝移植是失代偿终末期AIH患者的最后选择。移植免疫调节细胞，如调节性T细胞，在不远的将来可能成为有前景的治疗。
注意这种疾病，它的临床表现、治疗和进展，对肝脏病专家很重要，因为许多这种病例在以往都被忽略了。
原文阅读》》》Autoimmune Hepatitis in Asia
Autoimmune hepatitis (AIH) is a chronic inflammation of the liver caused by an abnormal autoimmune reaction against hepatocytes. While the prevalence of autoimmune hepatitis has been found to be 0.1-1.9/100,000 per year, 11-23% of all patients with chronic liver disease live with this condition.
The aetiology of AIH is currently unknown, but a host of environmental , genetic, and pathophysiological factors have been considered. The pathogenesis of AIH involves a loss of tolerance to hepatic self-antigens in a susceptible host. It is known that the disease involves an autoimmune reaction targeted at hepatocytes and that there is a high association with other autoimmune diseases, l ike type 1 diabetes, Graves disease, and proliferative glomerulonephritis.
The diagnosisis based on histological abnormalities, and characteristic clinical and biochemical findings, which include abnormal levels of serum globulins, and the presence of one or more characteristic auto-antibodies, such as ANA, SMA, and ant i -SLA. Interface hepatitis with abundant plasma cells in the infiltrate is a characteristic histological feature of AIH. The simplified criteria have high sensitivity and specificity for diagnosis of AIH in Asian patients. The revised original criteria have the complementary role to avoid the false negative diagnosis in atypical AIH patients.
Although the prevalence data are scarce, AIH has emerged as a major cause of non-viral chronic hepatitis in China. More and more l iver centresreport liver biopsy and serum autoantibody detection regularly. Alertness and awareness of hepatologists and histologic physicians on the histologic features of AIH contributes to more and more AIH patients being diagnosed in our clinics who were previously considered as cryptogenic chronic hepatitis cases.
Fortunately, AIH can be effectively managed with current therapies. Immunosuppressive treatment can attenuate hepatic inflammation, reversefibrosis, and eventually improve the patient’s prognosis and life quality.
AIH show a good response to immunosuppressive treatment , although patients with late -stage or severe disease are less likely to achieve remission. Liver transplantation is the last choice for AIH patients with decompensated end-stage disease. Transfer of regulatory immune cells such as regulatory T cells may become a potential therapy in the near future.
Awarenes s of this disease, its presentation , treatment , and progression, is crucial for hepatologists as many of these cases in the past have been overlooked.
