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

王婷婷; 鲁昌立; 凡小丽; 杨丽

doi:10.3969/j.issn.1001-5256.2017.11.026

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

DOI: 10.3969/j.issn.1001-5256.2017.11.026

四川大学华西医院消化内科

基金项目:

四川省科技厅支撑计划(2016FZ0062)；

详细信息

中图分类号: R575
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出版历程
- 收稿日期: 2017-05-02
- 出版日期: 2017-11-20

Clinical and pathological features of autoimmune hepatitis-primary biliary cholangitis overlap syndrome versus autoimmune hepatitis

Department of Gastroenterology, West China Hospital, Sichuan University

Research funding:

摘要

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、-5.40...
- 肝炎,自身免疫性 /
- 胆管炎,硬化性 /
- 病理学,临床 /
- 疾病特征
Abstract:
Objective To 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.Methods A 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.Results The 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) .Conclusion Although 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.
- hepatitis, autoimmune /
- cholangitis, sclerosing /
- pathology, clinical /
- disease attributes

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