Autoimmune liver disease
Autoimmune liver disease
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DOI：
10.1097/mog.0b013e3282f57268
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发表时间：
2008-05-01
影响因子：
2.5
通讯作者：
Czaja, Albert J.
中科院分区：
文献类型：
--
作者：
Czaja, Albert J.
综述的目的回顾提高自身免疫性肝炎的诊断和治疗的研究，并扩大其致病mechanism.Recent findingsBlack患者比白色患者有更先进的疾病和更差的结果。全基因组DNA微卫星技术已经确定了多个区域，可能赋予易感性或抵抗力的疾病。一条父母来源的X染色体的优先失活可能有利于女性的自身反应性。原因不明的急性和慢性肝炎对皮质类固醇治疗有反应，代表自身抗体阴性的自身免疫性肝炎。通过持续治疗直到所有特征消退以及早期识别具有终末期肝病模型的问题患者，可以改善结局。血清B细胞活化因子水平与肝损伤的实验室指标相关。他克莫司和霉酚酸酯是有前途的治疗有问题的患者，和抗原的非典型抗体的肝/肾微粒体的目标可能会导致诊断测试从头自身免疫性肝炎后肝transplantation.SummaryEthnic背景和遗传易感性影响自身免疫性肝炎的发生和结果。人类基因组中的易感性和耐药因素强调了该疾病的遗传复杂性。通过更好地使用当前方案和进一步评估特异性免疫抑制剂，可以改善结局。
Purpose of reviewTo review studies that improve the diagnosis and treatment of autoimmune hepatitis and extend understanding of its pathogenic mechanisms.Recent findingsBlack patients have more advanced disease and poorer outcomes than white patients. Genome-wide DNA microsatellite techniques have identified multiple regions that may confer susceptibility or resistance to the disease. Preferential inactivation of one parentally-derived X chromosome may favor autoreactivity in women. Acute and chronic hepatitis of undetermined cause can respond to corticosteroid therapy and represent autoantibody-negative autoimmune hepatitis. Outcomes can be improved by continuing therapies until resolution of all features and by early identification of problematic patients with the Model for End Stage Liver Disease. Serum levels of B-cell activating factor correlate with laboratory indices of liver injury. Tacrolimus and mycophenolate mofetil are promising therapies for problematic patients, and the antigenic targets of atypical antibodies to liver/kidney microsome may lead to diagnostic tests for de-novo autoimmune hepatitis after liver transplantation.SummaryEthnic background and genetic predisposition affect the occurrence and outcome of autoimmune hepatitis. Susceptibility and resistance factors across the human genome underscore the genetic complexity of the disease. Outcomes can be improved by better use of current regimens and further evaluation of action-specific immunosuppressive agents.