自身免疫性肝炎、原发性胆汁性胆管炎及其重叠综合征患者临床特征比较*

doi:10.3969/j.issn.1672-5069.2020.06.018

摘要/Abstract

摘要： 目的比较自身免疫性肝炎(AIH)、原发性胆汁性胆管炎(PBC)和AIH/PBC重叠综合征(AIH/PBC OS)患者临床特征的异同。方法 2017年12月～2019年2月南通市第三人民医院收治的92例自身免疫性肝病患者中包括AIH 35例、PBC 30例和AIH/PBC OS 27例,记录临床表现和血液化验资料并比较三组的异同。结果 AIH、PBC和AIH/PBC OS患者年龄、性别、体质指数和病程比较,无显著性差异(P>0.05);三组乏力、腹胀、纳差、发热和皮肤瘙痒发生率无显著性差异(P>0.05);PBC和AIH/PBC OS患者黄疸更深,血清ALP和GGT水平更高,而AIH患者血清ALT和AST水平更高 (P<0.05);AIH患者凝血功能指标TT显著长于其他两组(P<0.05);AIH患者外周血Hb水平显著低于其他两组(P<0.05);AIH患者血清ASMA阳性率为14.3%,显著高于其他两组的0.0%和0.0%(P<0.05),PBC和AIH/PBC OS患者血清AMA/AMA-M2阳性率分别为93.3%和92.6%,而AIH患者为0.0%(P<0.05);三组血清免疫球蛋白水平差异无统计学意义(P>0.05),三组合并甲状腺功能亢进、系统性红斑狼疮、干燥综合征、类风湿性关节炎、2型糖尿病和慢性肾小球肾炎方面差异,也无统计学意义(P>0.05)。结论 AIH、PBC和AIH/PBC OS患者具有相似的临床症状和体征,但血生化指标、自身抗体表现各有特征,了解这些特征对临床提高诊断和治疗水平将有很大的帮助。

关键词: 自身免疫性肝炎, 原发性胆汁性胆管炎, 重叠综合征, 临床特征

Abstract: Objective The aim of this study was to compare the clinical features of patients with autoimmune hepatitis (AIH), primary biliary cholangitis (PBC) and AIH/PBC overlap syndrome (AIH/PBC OS). Methods 92 patients with autoimmune liver diseases were admitted to the Third People's Hospital, Nantong city between December 2017 and February 2019, including AIH in 35, PBC in 30 and AIH/PBC OS in 27. The clinical data were compared. Results The ages, gender, body mass index and disease periods in patients with AIH, PBC and AIH/PBC were not significantly different(P>0.05); the incidences of clinical symptoms such as fatigue, abdominal distension, anorexia, fever and pruritus in the three groups were not much different(P>0.05); the jaundice was more common and higher serum ALP and GGT in patients with PBC and in with AIH/PBC OS, while the patients with AIH had higher serum ALT and AST levels (P<0.05); the TT was significantly longer in patients with AIH than in patients with PBC or AIH/PBC OS (P<0.05); the blodd Hb level in patients with AIH was significantly lower than in other two groups (P<0.05); the prevalence of serum ASMA in patients with AIH was 14.3%, significantly higher than 0.0% and 0.0% in other two groups(P<0.05), the positivity rates of AMA/AMA-M2 in patients with PBC and with AIH/PBC OS were 93.3% and 92.6%, while that in patients with AIH was 0.0%(P<0.05); serum immunoglobulin levels and the coincidence of hyperthyroidism, SLE, Sjogren's syndrome, rheumatoid arthritis, type two diabetes mellitus and chronic glomerulonephritis in the three groups were not significantly different(P>0.05). Conclusion The patients with AIH, PBC and AIH/PBC OS have sometimes similar clinical symptoms and signs, but there are obvious differences in biochemical and autoimmune antibodies, which might help the clinicians to deal with autoimmune liver diseases appropriately.

Key words: Autoimmune liver diseases, Autoimmune hepatitis, Primary biliary cholangitis, AIH/PBC overlap syndrome, Clinical features

张健荣, 顾尔莉, 王忠成, 施亚军. 自身免疫性肝炎、原发性胆汁性胆管炎及其重叠综合征患者临床特征比较^*[J]. 实用肝脏病杂志, 2020, 23(6): 829-832.

Zhang Jianrong, Gu Erli, Wang Zhongcheng, et al. Comparison of clinical characteristics of patients with autoimmune hepatitis, primary biliary cholangitis and AIH/PBC overlap syndrome[J]. Journal of Practical Hepatology, 2020, 23(6): 829-832.

参考文献

[1] Arndtz K, Hirschfield GM. The pathogenesis of autoimmune liver disease. Dig Dis, 2016, 34(4):327-333.
[2] Hadzic N, Hierro L. Autoimmune liver disease: novelties in management. Clin Res Hepatol Gastroenterol, 2014, 38(3):273-276.
[3] Kwo PY, Cohen SM, Lim JK. ACG clinical guideline: evaluation of abnormal liver chemistries. Am J Gastroenterol, 2017, 112(1):18-35.
[4] Muratori P, Fabbri A, Lalanne C, et al. Autoimmune liver disease and concomitant extrahepatic autoimmune disease. Eur J Gastroenterol Hepatol, 2015, 27(10):1175-1179.
[5] Xanthopoulos A, Starling RC, Kitai T, et al. Heart failure and liver disease: cardiohepatic interactions. JACC Heart Fail, 2019, 7(2):87-97.
[6] 刘红丽, 杨永峰, 熊清芳, 等.470例行肝穿刺活组织检查的不明原因肝病的病因分析.中华肝脏病杂志, 2019, 27(11):885-889.
[7] 马雄. 自身免疫性肝病的诊治:从共识到指南. 中华肝脏病杂志, 2016, 24(1):3-4.
[8] 蔺丽慧, 杨峻, 李甲勇, 等. 部分肥大细胞相关抗体及分子与自身免疫性肝病的关系.中华检验医学杂志, 2015, 38(9):617-621.
[9] 陈勇, 王绮夏, 马雄. 自身免疫性肝病2018年研究进展. 中华肝脏病杂志, 2019, 27(1):14-17.
[10] Selmi C, Generali E, Gershwin ME. Rheumatic manifestations in autoimmune liver disease. Rheum Dis Clin North Am, 2018, 44(1):65-87.
[11] Kur P, Kolasa-Wolosiuk A, Misiakiewicz-Has K, et al. Sex hormone-dependent physiology anddiseases of liver. Int J Environ Res Public Health, 2020, 17(8):E2620.
[12] Terziroli BB, Invernizzi P, Gershwin ME, et al. Skin manifestations associated with autoimmune liver diseases: a systematic review. Clin Rev Allergy Immunol, 2017, 53(3):394-412.
[13] Webb GJ, Hirschfield GM. Genetics of autoimmune liver disease: a brief summary for clinicians. Dig Dis, 2014, 32(5):e1-e6.
[14] Martinez CO, Diaz RG, Marin ZJ, et al. Autoimmune hepatitis - primary biliary cholangitis overlap syndrome. Long-term outcomes of a retrospective cohort in a university hospital. Gastroenterol Hepatol, 2018, 41(9):544-552.
[15] Wu HM, Sheng L, Wang Q, et al. Performance of transient elastography in assessing liver fibrosis in patients with autoimmune hepatitis-primary biliary cholangitis overlap syndrome. World J Gastroenterol, 2018, 24(6):737-743.
[16] 韩玉坤, 张文瑾, 陈菊梅, 等. 自身免疫性肝病临床与病理研究. 中华肝脏病杂志, 2001, 9(S1):82-84.
[17] Fujiwara K, Fukuda Y, Seza K, et al. Long-term observation of acute-onset autoimmune hepatitis presenting clinically and radiologically as acute hepatitis. Hepatol Int, 2018, 12(2):191-199.
[18] Adiga A, Nugent K. Lupus hepatitis and autoimmune hepatitis (lupoid hepatitis). Am J Med Sci, 2017, 353(4):329-335.
[19] Kerkar N, Chan A. Autoimmune hepatitis, sclerosing cholangitis, and autoimmune sclerosing cholangitis or overlap syndrome. Clin Liver Dis, 2018, 22(4):689-702.
[20] Park Y, Cho Y, Cho EJ, et al. Retrospective analysis of autoimmune hepatitis-primary biliary cirrhosis overlap syndrome in Korea: characteristics, treatments, and outcomes. Clin Mol Hepatol, 2015, 21(2):150-157.

自身免疫性肝炎、原发性胆汁性胆管炎及其重叠综合征患者临床特征比较^*

Comparison of clinical characteristics of patients with autoimmune hepatitis, primary biliary cholangitis and AIH/PBC overlap syndrome

PDF (PC)

可视化

摘要/Abstract

引用本文

使用本文

参考文献

相关文章 15

编辑推荐

Metrics

本文评价

[1]	李庆玲, 何树光, 易小明, 段婷娜, 周意. 雷公藤多甙联合异甘草酸镁治疗自身免疫性肝炎患者外周血淋巴细胞PD-1和PD-L1变化[J]. 实用肝脏病杂志, 2021, 24(1): 79-82.
[2]	孙文霞, 伍树芝. IgG4相关性硬化性胆管炎100例临床特征分析[J]. 实用肝脏病杂志, 2021, 24(1): 83-86.
[3]	王雨, 王楠, 王巧侠, 马清华, 潘新杰, 付建军. 利肝隆联合异甘草酸镁治疗自身免疫性肝炎患者疗效及其对血清趋化因子水平的影响^*[J]. 实用肝脏病杂志, 2020, 23(6): 821-824.
[4]	凌晶, 张友健, 宋雄峰. 自身免疫性肝炎患者血清趋化因子和GP73水平变化及其临床意义研究[J]. 实用肝脏病杂志, 2020, 23(6): 825-828.
[5]	刘松涛, 李娟, 于红卫, 侯维, 胡中杰. 9例以肝功能异常起病的恶性淋巴瘤患者临床特征分析[J]. 实用肝脏病杂志, 2020, 23(5): 650-653.
[6]	文琼花, 唐敏云, 魏玉, 陈少文. 血清腺苷脱氨酶和淀粉样蛋白A辅助诊断自身免疫性肝炎患者临床价值分析^*[J]. 实用肝脏病杂志, 2020, 23(4): 508-511.
[7]	程源, 张洋, 毛海洲, 段红光. 自身免疫性肝炎患者血清抗延胡索酸水合酶抗体检测及其临床意义探讨^*[J]. 实用肝脏病杂志, 2020, 23(4): 512-515.
[8]	李文渊, 叶超, 李磊, 方芳, 陈思, 王陆军, 张开光. 原发性胆汁性胆管炎患者血清自身抗体和临床特征分析^*[J]. 实用肝脏病杂志, 2020, 23(4): 516-519.
[9]	黄丽华, 杨正兵, 谭礼让. 熊去氧胆酸联合非诺贝特治疗PBC患者血清TGF-β、IFN-γ和IL-10水平变化^*[J]. 实用肝脏病杂志, 2020, 23(4): 520-523.
[10]	齐敬聪, 邢文静, 杜洋, 高辰玮. 熊去氧胆酸联合微生态制剂治疗原发性胆汁性胆管炎患者对肠道菌群的影响^*[J]. 实用肝脏病杂志, 2020, 23(4): 524-527.
[11]	杜颖,姜志红,吴雅玲. 雷公藤多甙联合异甘草酸镁治疗儿童自身免疫性肝炎疗效及其对血清IL-17和CXCL10水平的影响[J]. 实用肝脏病杂志, 2020, 23(3): 348-351.
[12]	吴志敏,杨永煌,卞相丽. 儿童和青少年自身免疫性肝炎患者外周血淋巴细胞亚群变化及其临床意义探讨[J]. 实用肝脏病杂志, 2020, 23(3): 352-355.
[13]	冯静,罗思剑,李靖,刘岚剑,邱国院,王翠,龚成丽. 不同类型自身免疫性肝病患者肝组织炎症因子表达的变化[J]. 实用肝脏病杂志, 2020, 23(3): 356-359.
[14]	朱步坤,李刚,张玮. 中西医药治疗自身免疫性肝病研究进展[J]. 实用肝脏病杂志, 2020, 23(3): 453-456.
[15]	张玉玲, 周瑾, 董宏伟. 超声点定量弹性成像技术对AIH和PBC患者肝纤维化诊断价值分析^*[J]. 实用肝脏病杂志, 2020, 23(2): 231-235.