原发性胆汁性胆管炎患者临床特征、自身抗体特点和预后分析*

doi:10.3969/j.issn.1672-5069.2026.05.012

摘要/Abstract

摘要： 目的分析总结原发性胆汁性胆管炎(PBC)的临床特征、血清自身抗体和治疗应答情况,特别比较血清抗gp210抗体阳性与阴性患者的临床差异。方法 2020年3月～2024年12月我院诊治的PBC患者60例,均接受熊去氧胆酸(UDCA)长期治疗。采用电化学发光法检测血清免疫球蛋白水平,使用德国欧蒙试剂和全自动免疫印迹法检测血清自身抗体,使用FibroScan行肝脏硬度检测(LSM)。结果本组患者首发症状为乏力占46.7%,皮肤瘙痒占31.7%,黄疸占21.7%,右上腹部不适占11.7%,腹水占5.0%,脾肿大占3.3%,消化道症状占5.0%,合并干燥综合征占23.3%,系统性红斑狼疮占6.7%,类风湿性关节炎占5.0%;血清AMA-M2阳性占91.7%,AMA阳性占81.7%,抗SLA/LP阳性占3.3%,抗LKM-1阳性占1.7%,抗gp210阳性占26.7%,抗Sp100阳性占15.0%,抗LC-1阳性占3.3%;16例血清抗gp210阳性组血清ALP、总胆红素、IgM、LSM和Mayo风险评分分别为(295.0±82.5)U/L、(24.5±10.3)μmol/L、(4.2±1.1)g/L、(14.1±3.8)kPa和(5.9±1.2),均显著高于44例血清抗gp210阴性组【分别为(210.5±65.8)U/L、(15.7±7.6)μmol/L、(3.7±1.0)g/L、(10.8±3.0)kPa和(4.7±1.0),P<0.05】,而对UDCA治疗应答率为43.7%,显著低于血清抗gp210阴性组的77.3%(P<0.05);随访26个月,血清抗gp210阳性组肝硬化、肝功能失代偿和肝移植/死亡发生率分别为43.7%、37.5%和18.7%,均显著高于血清抗gp210阴性组的20.4%、11.4%和4.5%(P<0.05)。结论血清抗gp210抗体阳性的PBC患者可能预示着更为严重的临床病情和较差的预后,值得深入研究。

关键词: 原发性胆汁性胆管炎, 抗gp210抗体, 抗线粒体抗体, 熊去氧胆酸, 治疗

Abstract: Objective The aim of this study was to investigate clinical feature, serum autoantibody profiles and prognosis of patients with primary biliary cholangitis (PBC). Methods 60 patients with PBC were encountered in our hospital between March 2020 and December 2024, and all received long-term oral ursodeoxycholic acid (UDCA) treatment. Serum IgM, autoantibody profiles were detected routinely, and liver stiffness measurement (LSM) was measured by Fibroscan. Results As for initial symptoms in our 60 patients with PBC, fatigue accounted for 46.7%, itching for 31.7%, jaundice for 21.7%, right upper quadrant discomfort for 11.7%, ascites for 5.0%, splenomegaly for 3.3% and gastrointestinal symptoms for 5.0%, and concomitant with SjÏgren's syndrome for 23.3%, systemic lupus erythematosus for 6.7% and rheumatoid arthritis for 5.0%; serum AMA-M2 positive rate accounted for 91.7%, AMA for 81.7%, anti-SLA/LP for 3.3%, anti-LKM-1 for 1.7%, anti-gp210 for 26.7%, anti-Sp100 for 15.0% and anti-LC-1 for 3.3%; serum ALP, total serum bilibubin, IgM, LSM and Mayo’s score in 16 patients with serum anti-gp210 positive were (295.0±82.5)U/L, (24.5±10.3)μmol/L, (4.2±1.1)g/L, (14.1±3.8)kPa and (5.9±1.2), all significantly higher than [(210.5±65.8)U/L, (15.7±7.6)μmol/L, (3.7±1.0)g/L, (10.8±3.0)kPa and (4.7±1.0), respectively, P<0.05], while complete response rate to UDCA therapy was 43.7%, significantly lower than 77.3%(P<0.05) in 44 patients with serum anti-gp210 negative; by end of medium 26 month of follow-up, the incidences of liver cirrhosis, decompensated liver functions and liver transplantation/death in patients with serum anti-gp210 positive were 43.7%, 37.5% and 18.7%, all much higher than 20.4%, 11.4% and 4.5%(P<0.05) in those with serum anti-gp210 negative. Conclusion Serum anti-gp210 antibody positive might hints even more clinical severity of disease, and clinicians should take more attention to them.

Key words: Primary biliary cholangitis, Anti-gp210 antibody, Anti-mitochondrial antibody, Ursodeoxycholic acid, Therapy

赵先群, 闫斌斌, 李德洋, 田春阳, 张向东. 原发性胆汁性胆管炎患者临床特征、自身抗体特点和预后分析^*[J]. 实用肝脏病杂志, 2026, 29(5): 685-688.

Zhao Xianqun, Yan Binbin, Li Deyang, et al. Clinical feature, serum autoantibody profiles and prognosis of patients with primary biliary cholangitis[J]. Journal of Practical Hepatology, 2026, 29(5): 685-688.

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原发性胆汁性胆管炎患者临床特征、自身抗体特点和预后分析^*

Clinical feature, serum autoantibody profiles and prognosis of patients with primary biliary cholangitis

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