肝移植术后新发自身免疫性肝炎单中心36例临床分析

Clinical analysis of 36 cases of de novo autoimmune hepatitis after liver transplantation in a single center

目的探讨肝移植术后新发自身免疫性肝炎（DNAH）的临床表现、诊疗特点及转归。方法回顾性分析2002年6月至2014年8月1601例肝移植受者中36例发生DNAH受者的临床特征、生化免疫学指标、病理学检查结果及转归。按照2008年公布的最新自身免疫性肝炎的诊断积分系统评分≥7分者13例，=6分者6例，〈6分者17例。结果DNAH临床症状表现复杂多样、缺乏特异性，尤以丙氨酸转氨酶（ALT）、天冬氨酸转氨酶（AsT）升高最明显；36例DNAH受者的血清抗核抗体阳性率为86．1％（31／36）、抗线粒体抗体阳性率为2．8％（1／36），未检测到其他自身抗体；IgG阳性率为61．1％（22／36）。肝组织中出现汇管区和小叶内淋巴细胞和（或）浆细胞浸润者共17例，界面性肝炎者8例，肝细胞玫瑰花结样改变1例。36例受者经治疗后，29例（69．2％）出现完全应答，6例（16．7％）出现部分应答，1例（2．80）在治疗期间出现丙型病毒性肝炎复发，治疗失败。29例完全应答受者中，27例（75．0%）持续缓解，2例（5．6％）复发。结论肝移植术后发生DNAH的受者，其血清抗核抗体、IgG及肝组织病理阳性率均较高，但早期确诊率低；绝大部分DNAH对单用激素或联合硫唑嘌呤治疗反应良好，只要早期发现、及时治疗能取得良好效果。

Objective To explore the clinical manifestations, diagnosis and treatment and prognosis of De novo autoimmune hepatitis （DNAH） after liver transplantation. Method The clinical features, biochemical and immunological parameters, pathological changes and prognosis of 36 cases of DNAH after liver transplantation from June 2002 to August 2014 were retrospectively analyzed. Result The clinical manifestations of DNAH were multiplex and lack of specificity, especially the ALT and AST increased significantly. The positive rate of serum antinuclear antibodies in 36 cases of DNAH was 86. 1% （31/36）, that of anti-mitochondrial antibody was 2.8% （1/36）, and other autoantibodies were not detected. The positive rate of IgG was 61.1% （22/36）. Infiltration of periportal and lobular lymphocytes and/or plasma cells in liver tissues was found in 17 cases （58. 6%）, interface hepatitis in 8 cases （27. 6%）, and liver cell rosette-like changes in l case （3.4%）. In 36 cases of DNAH, 29 patients obtained a complete response （69. 2%）, 6 patients obtained a partial response （16. 7%）, and 1 patient （2. 8%） with hepatitis C relapse during treatment had failure treatment. Among 29 cases of complete response, 27 cases （75.0%） achieved sustained remission, and recurrence occurred in 2 cases （5.6%）. Conclusion The positive rate of serum antinuclear antibodies, IgG and abnormal liver pathological changes in 36 cases of DNAH was high, but the early diagnostic rate was low. The treatment responses for most patients with DNAH alone or in combination with azathioprine hormone were satisfactory, as long as the early detection and timely treatment could be given.