首 页   本刊简介  编委会  审稿专家  在线期刊  写作规范  广告合作  联系我们
您现在的位置:首页 => 在线期刊 => 11期原发性胆汁性胆管炎 => 其他 =>自身免疫性肝炎-原发性胆汁性..
自身免疫性肝炎-原发性胆汁性胆管炎重叠综合征的临床及病理学特征分析
Clinical and pathological features of autoimmune hepatitis-primary biliary cholangitis overlap syndrome versus autoimmune hepatitis
文章发布日期:2017年10月11日  来源:  作者:王婷婷,鲁昌立,凡小丽,等  点击次数:153次  下载次数:28次

调整字体大小:

(此处下载失败可以在在线预览处保存副本或者右键另存为)

【摘要】:目的自身免疫性肝炎-原发性胆汁性胆管炎重叠综合征(AIH-PBC OS)的诊断仍然是目前尚未解决的难点,对AIH-PBC OS与AIH的临床与病理特征进行比较分析,以加强对AIH-PBC OS的认识,提高AIH-PBC OS的早期诊断率。方法前瞻性收集2015年1月20日-2016年11月28日于四川大学华西医院就诊且经肝穿刺确诊的AIH-PBC OS患者(50例)和单纯AIH患者(57例)的临床特征、生化免疫学指标、自身抗体和肝组织病理学表现等方面,比较AIH-PBC OS与AIH的不同特点。实验室指标等计量资料2组间比较采用非参数秩和检验;临床特征、肝组织病理学表现等计数资料2组间比较采用χ2检验。结果AIH-PBC OS组发病年龄低于AIH组(Z=-1.975,P=0048),男女性别比无明显差异。AIH-PBC OS患者较AIH患者更易出现瘙痒症状(22.0% vs 7.0%,χ2=4.960,P=0.026)。AIH-PBC OS组ALP、GGT和IgM水平以及AMA的阳性率均高于AIH组(Z=-6.250、-4.789、-5.407、χ2=26.811,P值均<0001),而血清ALT、AST和IgG的水平则低于AIH组(Z=-2.916、-3381、-2.285,P=0.004、0.001、0.022)。肝穿刺活组织检查AIH-PBC OS组患者胆管损伤、胆管缺失、肉芽肿样病变和胆汁淤积的发生率均高于AIH组(χ2=25.612、15.953、6.490、10042,P<0.001、<0.001、0.001、0.002)。结论AIH-PBC OS虽然同时具备AIH和PBC的特点,但并非AIH与PBC的简单叠加。瘙痒,ALP、GGT、IgM水平显著升高,AMA阳性,病理学表现出的炎症性胆管病变和胆汁淤积均对AIH-PBC OS的诊断有提示作用。由于AIH-PBC OS的肝脏炎症损伤程度和自身免疫异常程度总体可能较AIH轻,故在AIH-PBC OS诊断时应考虑适当放宽巴黎标准中ALT、IgG的水平,以防因漏诊而影响治疗方案的选择和患者预后。
【Abstract】:ObjectiveTo investigate the clinical and pathological features of autoimmune hepatitis (AIH)-primary biliary cholangitis (PBC) overlap syndrome (AIH-PBC OS) versus AIH, since it is still difficult to diagnose AIH-PBC OS in clinical practice, and to enhance the knowledge of AIH-PBC OS and improve its early diagnostic rate. MethodsA prospective study was performed for the clinical data, biochemical and immunological markers, autoantibody, and liver pathology of 50 patients with AIH-PBC OS confirmed by liver biopsy who visited West China Hospital, Sichuan University, from January 20, 2015 to November 28, 2016 and 57 patients with AIH alone, in order to compare the features of AIH-PBC OS and AIH. Continuous data such as laboratory markers were expressed as median M (inter-quartile range) and the nonparametric rank sum test was used for comparison of these data between two groups; categorical data including clinical features、histopathological manifestations were expressed as rates and the chi-square test was used for comparison of these data between two groups. ResultsThe AIH-PBC OS group had a significantly lower age of onset than the AIH group (Z=-1.975, P=0048), while there was no significant difference in male/female sex ratio between the two groups. The AIH-PBC OS group had a significantly higher proportion of patients with pruritus than the AIH group (22.0% vs 7.0%, χ2=4.960, P=0.026). Compared with the AIH group, the AIH-PBC OS group had significantly higher levels of alkaline phosphatase (ALP), gamma-glutamyl transpeptidase (GGT), and immunoglobulin M (IgM) (Z=-6.250, -4.789, and -5.407, all P<0.001), a significantly higher positive rate of antimitochondrial antibody (AMA) (χ2=26.811, P<0.001), and significantly lower serum levels of alanine aminotransferase (ALT), aspartate aminotransferase, and immunoglobulin G (IgG) (Z=-2.916, -3.381, and -2.285, P=0.004, 0.001, and 0.022). The AIH-PBC OS group had significantly higher incidence rates of bile duct injury, bile duct loss, granulomatous disease, and cholestasis than the AIH group (χ2=25.612, 15.953, 6.490, and 10.042, P<0.001, <0.001, 0.001, and 0.002). ConclusionAlthough AIH-PBC OS has the features of both AIH and PBC, it is not a simple overlap between AIH and PBC. Pruritus, significant increases in the levels of ALP, GGT, and IgM, positive AMA, and inflammatory bile duct disease and cholestasis may have implications for the diagnos of AIH-PBC OS. Since AIH-PBC OS may have milder liver inflammatory injury and autoimmune abnormalities than AIH, the levels of ALT and IgG in Paris criteria can be broadened during the diagnosis of AIH-PBC OS, in order to prevent missed diagnosis and avoid affecting the selection of treatment regimen and patients′ prognosis.
【关键字】:肝炎, 自身免疫性; 胆管炎, 硬化性; 病理学, 临床; 疾病特征
【Key words】:hepatitis, autoimmune; cholangitis, sclerosing; pathology, clinical; disease attributes
【引证本文】:王婷婷, 鲁昌立, 凡小丽, 等. 自身免疫性肝炎-原发性胆汁性胆管炎重叠综合征的临床及病理学特征分析[J]. 临床肝胆病杂志, 2017, 33(11): 2179-2185.

地址:长春市东民主大街519号《临床肝胆病杂志》编辑部 邮编:130061 电话:0431-88782542/3542
临床肝胆病杂志 版权所有 Copyright © 2009 - 2013 Lcgdbzz.org. All Rights Reserv 吉ICP备10000617号

吉公网安备 22010402000041号