IgG4相关性自身免疫性肝炎的临床和病理特征分析

Clinical and pathological features of IgG4-related autoimmune hepatitis

目的探讨IgG4相关性自身免疫性肝炎(IgG4-AIH)的临床表现、血清学特点和组织病理学特征。方法选取2014年7月至2022年6月昆明医科大学附属延安医院经肝组织病理学检查确诊的38例自身免疫性肝炎(AIH)患者,观察临床表现和血清学特点,收集肝组织标本,行HE染色和IgG4免疫组织化学染色,观察其组织学特征和IgG4阳性浆细胞浸润情况。结果38例AIH患者中,有7例血清IgG4水平明显升高,肝组织IgG4染色阳性,患者归为IgG4-AIH组,另31例归为经典型AIH组。两组患者均以黄疸、乏力、纳差为主要临床表现,肝功能异常以ALT、AST升高为主,两组患者无显著差异。IgG4-AIH组患者ANA阳性为6/7,而经典型AIH组患者ANA阳性率为83.8%(26/31),IgG-AIH组血清IgG水平明显升高为(33.2±7.2),而经典型AIH组IgG水平为(26.5±5.3),差异有统计学意义(t=2.828,P=0.008)。IgG4-AIH组患者肝脏组织学炎症以G3、G4期为主,而经典型AIH以G1、G2为主,两者差异显著,两组患者纤维化水平无明显差异。结论IgG4-AIH患者血清及肝组织IgG4水平显著升高,且肝脏病理检测提示炎症活动更明显,但IgG4-AIH与经典型AIH在临床表现、生化学指标方面无显著差异。

Objective To investigate the clinical presentations,serological features,and histopathological characteristics of IgG4-related autoimmune hepatitis(IgG4-AIH).Methods We collected and analyzed the clinical presentations and serological characteristics of 38 patients diagnosed with autoimmune hepatitis(AIH)based on liver tissue histopathology.Liver tissue samples were ssubjected to HE staining as well as immunohistochemical staining for IgG4 to evaluate histological features and the extent of IgG4-positive plasma cell infiltration.Results Among the 38 AIH patients,7 exhibited elevated serum IgG4 levels and positive liver tissue IgG4 staining,thus categorizing them into the IgG4-AIH group.The remaining 31 cases were considered part of the classic AIH group.Common symptoms observed in both groups included jaundice,fatigue,and anorexia,accompanied by liver function abnormalities characerized by elevated levels of ALT and AST.Autoantibody ANA was detected in 85.7%(6/7)of IgG4-AIH patients,and in 83.8%(26/31)of classic AIH patients.The serum IgG level was significantly higher in the IgG-AIH group(33.2±7.2)compared to the classic AIH group(26.5±5.3).Liver histological inflammation in the IgG4-AIH group was predominantly categorized as G3 and G4,while in the classic AIH group,it mainly fell under G1 and G2.The differences observed between the two groups were statistically significant.Conclusion Serum and liver tissue IgG4 levels were notably elevated in IgG4-AIH patients,with more pronounced inflammatory activity observed in liver pathological examination.Nevertheless,there were no significant difference in clinical manifestations and biochemical indicators between IgG4-AIH and classical AIH patients.