PBC和PBC-AIH重叠综合征患者外周血树突状细胞及其亚群变化初步研究*

doi:10.3969/j.issn.1672-5069.2025.04.023

摘要/Abstract

摘要： 目的探讨原发性胆汁性胆管炎(PBC)和PBC-自身免疫性肝炎(AIH)重叠综合征(OS)患者外周血树突状细胞(DC)及其亚群变化及其临床意义。方法 2020年1月～2024年1月南通市第三人民医院诊治的56例PBC患者和20例PBC-AIH OS患者,给予熊去氧胆酸(UDCA)或UDCA联合免疫抑制剂治疗12个月。使用流式细胞术检测外周血浆细胞样DC(pDC)、经典样DC(cDC)、1型经典样DC(cDC1)和2型经典样DC(cDC2)百分比。结果 PBC-AIH OS患者血清ALT、AST、TBIL、ALB、GLO、TBA、IgG和IgA水平显著高于PBC患者(P<0.05);PBC患者外周血cDC、pDC、cDC1和cDC2水平分别为1.1(0.6,1.9)%、0.2(0.1,0.4)%、0.004(0.003,0.010)%和0.2(0.1,0.4)%,均显著高于PBC-AIH OS患者【分别为0.7(0.4,1.0)%、0.1(0.1,0.2)%、0.004 (0.0004,0.007)%和0.2(0.1,0.2)%,P<0.05】;在治疗12个月末,应用巴黎标准评估,PBC-AIH OS患者完全应答12例(60.0%),PBC患者完全应答47例(83.9%);完全应答的PBC患者外周血cDC和cDC2百分比分别为(1.4±1.0)%和0.3(0.2,0.4)%,均显著高于未应答组【分别为(0.6±0.4)%和0.2(0.1,0.2)%,P<0.05】;完全应答的PBC-AIH OS患者cDC和cDC1百分比分别为0.7(0.5,1.0)%和(0.01±0.002)%,显著高于未应答组【分别为0.4(0.2,0.5)%和(0.0003±0.001)%,P<0.05】。结论 PBC和PBC-AIH OS患者外周血树突状细胞及其亚群可能发生了显著的变化,并可能影响治疗应答,值得深入研究。

关键词: 原发性胆汁性胆管炎, 原发性胆汁性胆管炎-自身免疫性肝炎重叠综合征, 树突状细胞, 熊去氧胆酸, 标准免疫抑制治疗, 应答

Abstract: Objective The aim of this study was to investigate the clinical implication of peripheral blood dendritic cells (DC) and their subsets in patients with primary biliary cholangitis (PBC) and PBC-autoimmune hepatitis overlap syndrome (PBC-AIH OS) . Methods 56 patients with PBC and 20 patients with PBC-AIH OS were encountered in Nantong Third People's Hospital between January 2020 and January 2024, receiving ursodeoxycholic acid (UDCA) or UDCA with immunosuppressant combination treatment for 12 months. Percentages of peripheral blood dendritic cell subpopulations, including plasmacytoid dendritic cells (pDC), conventional dendritic cells (cDC), conventional type 1 dendritic cells (cDC1) and conventional type 2 dendritic cells (cDC2) were detected by flow cytometry. Results Serum ALT, AST, bilirubin, albumin, globulin, bile acid, IgG and IgA levels in patients with PBC-AIH OS were significantly higher than those in patients with PBC (P<0.05); percentages of peripheral blood cDC, pdc, cDC1and cDC2 cells in patients with PBC were 1.1 (0.6, 1.9) %, 0.2 (0.1, 0.4) %, 0.004 (0.003, 0.010) % and 0.2 (0.1, 0.4) %, is significantly higher than [0.7 (0.4, 1.0) %, 0.1 (0.1, 0.2) %, 0.004 (0.0004, 0.007) % and 0.2 (0.1, 0.2) %, P< 0.05)] in patients with PBC- AIH OS; by end of 12-month treatment, complete response (CR) by Paris's criteria was found in 12 cases 60.0%)in patients with PBC-AIH OS, and in 47 cases (83.9%) in patients with PBC; percentages of cDC and cDC2 cells in CR patients with PBC were (1.4±1.0)% and 0.3(0.2,0.4)%, both significantly higher than [(0.6±0.4)% and 0.2(0.1,0.2)%, respectively P<0.05] in non-responders; percentages of cDC and cDC1 cells in CR patients with PBC-AIH OS were 0.7(0.5, 1.0)% and (0.01±0.002)%, both much higher than [0.4(0.2, 0.5)% and(0.0003±0.001)%, respectively, P<0.05] in non-responders. Conclusion DCs and their subpopulations change greatly, which might impact response of UDCA and/or immunosuppressant combination treatment, and needs further investigation.

Key words: Primary biliary cholangitis, Primary biliary cholangitis-autoimmune hepatitis overlap syndrome, Ursodeoxycholic acid, Immunosuppressant, Dendritic cells, Response

王燕燕, 周桐桐, 李娜, 卞兆连. PBC和PBC-AIH重叠综合征患者外周血树突状细胞及其亚群变化初步研究^*[J]. 实用肝脏病杂志, 2025, 28(4): 569-572.

Wang Yanyan, Zhou Tongtong, Li Na, et al. A preliminary study on changes of peripheral blood dendritic cells and their subsets in patients with PBC and PBC-AIH overlap syndrome[J]. Journal of Practical Hepatology, 2025, 28(4): 569-572.

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PBC和PBC-AIH重叠综合征患者外周血树突状细胞及其亚群变化初步研究^*

A preliminary study on changes of peripheral blood dendritic cells and their subsets in patients with PBC and PBC-AIH overlap syndrome

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