Ferri Priscila M, Simões E Silva Ana C, Torres Karen C L, Silva Soraya L C, Aquino Diego J Q, Ferreira Maria L M, Fagundes Eleonora D T, Miranda Débora M, Ferreira Alexandre R
Department of Pediatrics, UFMG.
Hospital das Clínicas da UFMG.
J Pediatr Gastroenterol Nutr. 2018 Feb;66(2):204-211. doi: 10.1097/MPG.0000000000001783.
The pathophysiology of autoimmune hepatitis (AIH) may involve the activation of immune cells and changes in the expression of cellular markers. The aim of the present study was to characterize the immunophenotype markers of lymphocytes and monocytes in the peripheral blood of children and adolescents with type 1 AIH and AIH overlap with sclerosing cholangitis (overlap syndrome [OS]).
This is a cross-sectional study of 20 children and adolescents diagnosed with type 1 AIH and 19 with OS. Fifteen healthy subjects were included as controls. Flow cytometric analysis was used to identify markers of inflammation and autoimmunity.
The total number of CD4 T cells was higher in the AIH patients compared with the controls. The number of CD4 T cells expressing CCR3 and CD28 was higher in the AIH group than in the control group. CD45RO was more highly expressed in the AIH group, whereas CD45RA was more highly expressed in the OS group. In regard to CD8 T lymphocytes, the CCR3 expression was higher in both groups of patients. Patients with OS had the highest expression of CD45RA and CD25. In monocytes, human leukocyte antigen DR (HLA-DR) was less expressed in both groups of patients.
Complex phenotype features may be involved in the pathophysiology of AIH, accounting for changes in immune system regulation mechanisms. In conclusion, even after good response to treatment, patients still have immune activity signals at the cellular level.
自身免疫性肝炎（AIH）的病理生理学可能涉及免疫细胞的激活和细胞标志物表达的变化。本研究的目的是表征1型AIH以及AIH合并硬化性胆管炎（重叠综合征[OS]）的儿童和青少年外周血中淋巴细胞和单核细胞的免疫表型标志物。
这是一项横断面研究，纳入了20例诊断为1型AIH的儿童和青少年以及19例OS患者。纳入15名健康受试者作为对照。采用流式细胞术分析来识别炎症和自身免疫的标志物。
与对照组相比，AIH患者的CD4 T细胞总数更高。AIH组中表达CCR3和CD28的CD4 T细胞数量高于对照组。CD45RO在AIH组中表达更高，而CD45RA在OS组中表达更高。关于CD8 T淋巴细胞，两组患者的CCR3表达均更高。OS患者的CD45RA和CD25表达最高。在单核细胞中，两组患者的人类白细胞抗原DR（HLA-DR）表达均较低。
复杂的表型特征可能参与AIH的病理生理学，这解释了免疫系统调节机制的变化。总之，即使在治疗反应良好后，患者在细胞水平上仍有免疫活性信号。
