Clinical Research Center, National Hospital Organization Nagasaki Medical Center, Omura, Japan.
Department of Hepatology, Nagasaki University Graduate School of Biomedical Sciences, Omura, Japan.
Clin Mol Hepatol. 2021 Jan;27(1):58-69. doi: 10.3350/cmh.2020.0189. Epub 2020 Dec 10.
Autoimmune hepatitis (AIH) is an immunoinflammatory chronic liver disease with dynamic and rather heterogeneous disease manifestations. A trend of increasing prevalence of AIH has been observed worldwide, along with a relative increase in the percentage of male patients. AIH is characterized and diagnosed based on serum biochemistry and liver histology: elevated aminotransferases and serum immunoglobulin G (IgG), the presence of serum anti-nuclear antibody or anti-smooth muscle antibody, and interface lympho-plasmacytic hepatitis. Clinical manifestations differ among disease subtypes with distinct time-frames, i.e., AIH with a chronic insidious onset, and acute-onset AIH (the diagnosis of which is often challenging due to the lack of typical serum findings). The absence of disease-specific biomarkers or histological findings may expand the disease phenotype into drug-induced AIH-like liver injury. Corticosteroids and azathioprine are recommended first-line treatments for AIH. The complete normalization of aminotransferases and serum IgG is an essential treatment response to ensure long-term overall survival. An incomplete response or intolerance to these drugs is considered an indication for second-line treatment, especially with mycophenolate mofetil. Life-long maintenance treatment is required for the majority of patients, but the few who achieve prolonged and stringent biochemical remission with lower alanine aminotransferase and IgG within the normal range may be able to discontinue the medications. In the future, the quality of life of AIH patients should be managed by personalized medicine, including the appropriate selection and dosing of first-line therapy and perhaps alternating with potential therapeutics, and the prediction of the success of treatment withdrawal.
自身免疫性肝炎(AIH)是一种免疫炎症性慢性肝病,具有动态和相当异质性的疾病表现。全球范围内观察到 AIH 的患病率呈上升趋势,同时男性患者的比例相对增加。AIH 的特征和诊断基于血清生化和肝组织学:转氨酶和血清免疫球蛋白 G(IgG)升高,存在血清抗核抗体或抗平滑肌抗体,以及界面淋巴浆细胞性肝炎。不同疾病亚型的临床表现存在差异,具有不同的时间框架,即慢性隐匿性发病的 AIH 和急性发病的 AIH(由于缺乏典型的血清发现,其诊断常常具有挑战性)。缺乏疾病特异性生物标志物或组织学发现可能会将疾病表型扩展为药物诱导的类似 AIH 的肝损伤。皮质类固醇和硫唑嘌呤被推荐为 AIH 的一线治疗药物。转氨酶和血清 IgG 完全正常是确保长期总体生存的必要治疗反应。对这些药物无反应或不耐受被认为是二线治疗的指征,尤其是霉酚酸酯。大多数患者需要终身维持治疗,但少数患者在正常范围内实现了较低的丙氨酸转氨酶和 IgG 的延长和严格的生化缓解,可能能够停止用药。未来,AIH 患者的生活质量应通过个性化医疗来管理,包括一线治疗的适当选择和剂量调整,以及可能与潜在治疗药物交替使用,并预测治疗停药的成功。