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自身免疫性肝病并发腹腔淋巴结肿大的临床特征分析
Clinical features of autoimmune liver disease complicated by abdominal lymphadenectasis
文章发布日期:2017年10月11日  来源:  作者:张璐,刘丽娜  点击次数:646次  下载次数:111次

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【摘要】:目的探讨自身免疫性肝病(AILD)合并腹腔淋巴结肿大(LA)患者的临床特征及LA在AILD诊断中的临床意义。方法回顾性分析2005年1月-2016年4月大连医科大学附属第一医院收治的252例AILD患者的病例资料,其中自身免疫性肝炎(AIH)患者52例,原发性胆汁性胆管炎(PBC)患者174例,AIH-PBC 重叠综合征患者26例,获得随访病例78例。按照是否合并LA分为LA组70例,无淋巴结肿大(NLA)组182例。一般资料、实验室检查指标、影像学检查及随访结果符合正态分布的计量资料以均数±标准差(x±s)表示,组间比较采用t检验;不符合正态分布则以中位数(最小值~最大值)表示,组间比较采用Wilcoxon秩和检验;计数资料以率表示,组间比较采用χ2检验、矫正χ2检验或Fisher确切概率法分析。结果2组在年龄、性别比例、因体检发现肝功能异常而诊断的比例及合并其他自身免疫性疾病的发生率间差异均无统计意义。AIH、PBC及AIH-PBC重叠综合征腹腔LA的发生率分别为212%(11/52),26.4%(46/174),500%(13/26),AIH-PBC重叠综合征腹腔LA发生率高于AIH和PBC(χ2=6.771,P=0.009; χ2=6039,P=0014)。LA组ALP、GGT水平高于NLA组,2组比较差异有统计学意义(Z值分别为2944、3.169,P=0003、0.002);PBC患者中,LA组GGT水平高于NLA组(Z=2.136,P=0.033);AIH-PBC重叠综合征患者中,LA组TBil明显高于NLA组(Z=2.121,P=0.035);AIH患者中,LA组与NLA组各项检查指标改变无明显差异。LA组影像学异常发生率较NLA组高(97.1% vs 81.9%,χ2=9.863,P=0.002)。获得随访病例78例,中位随访时间18个月,LA组6例获得完全缓解(23.1%),7例不完全应答(26.9%),1例复发(3.8%),12例治疗失败(462%);NLA组21例完全缓解(40.4%),17例不完全应答(327%),7例复发(13.5%),7例治疗失败(13.5%),LA组与NLA组药物治疗反应组间分布不同(Z=2.406,P=0.016)。结论AIH-PBC重叠综合征 伴腹腔LA的发生率高于AIH及PBC,伴有LA的AILD胆汁淤积及肝功能损伤更为明显,药物治疗反应较差,提示合并LA可能为疾病严重程度估计及预后判断的一个指标。
【Abstract】:ObjectiveTo investigate the clinical features of autoimmune liver disease (AILD) complicated by abdominal lymphadenectasis (LA), as well as the clinical significance of LA in AILD. MethodsA retrospective analysis was performed for the clinical data of 252 patients who were admitted to The First Affiliated Hospital of Dalian Medical University from January 2005 to April 2016, and among these patients, 52 had autoimmune hepatitis (AIH), 174 had primary biliary cholangitis (PBC), and 26 had AIH-PBC overlap syndrome (AIH-PBC OS). A total of 78 patients underwent follow-up. According to the presence or absence of LA, these patients were divided into LA group with 70 patients and non-LA (NLA) group with 182 patients. As for general information, laboratory markers, imaging findings, and follow-up results, the normally distributed continuous data were expressed as mean±SD, and the t-test was used for comparison between groups; the non-normally distributed continuous data were expressed as median, and the Wilcoxon rank sum test was used for comparison between groups. The categorical data were expressed as rates, and the chi-square test, the corrected chi-square test, or the Fisher′s exact test was used for comparison of these data between groups. ResultsThere were no significant differences between the two groups in age, sex ratio, proportion of patients who were diagnosed due to abnormal liver function found by physical examination, and incidence rates of other autoimmune diseases. The incidence rate of abdominal LA was 22% (11/52) in the AIH group, 26.4% (46/174) in the PBC group, and 50% (13/26) in the AIH-PBC OS group, and the AIH-PBC OS group had a significantly higher incidence rate than the AIH group and the PBC group (χ2=7.693, P=0.021). The LA group had significantly higher levels of alkaline phosphatase and gamma-glutamyl transpeptidase (GGT) than the NLA group (Z=2.944 and 3.169, P=0.003 and 0.002). For the patients with PBC, the LA group had a significantly higher level of GGT than the NLA group (Z=2.136, P=0.033); for the patients with AIH-PBC OS, the LA group had a significantly higher level of total bilirubin than the NLA group (Z=2.121, P=0.035); for the patients with AIH, there were no significant differences in these indices between the LA group and the NLA group. The LA group had a higher incidence rate of abnormal imaging findings than the NLA group (97.1% vs 81.9%, χ2=9.863, P=0.002). A total of 78 patients were followed up with a median follow-up time of 18 months. Of all patients in the LA group, 6 (23.1%) achieved complete remission, 7 (26.9%) achieved incomplete response, 1 (3.8%) experienced recurrence, and 12 (46.2%) experienced treatment failure; of all patients in the NLA group, 21 (404%) achieved complete remission, 17 (32.7%) achieved incomplete response, 7 (13.5%) experienced recurrence, and 7(13.5%) experienced treatment failure; there was a significant difference in the distribution of treatment outcomes between the LA group and the NLA group (Z=2.406, P=0.016). ConclusionPatients with AIH-PBC OS have a higher incidence rate of abdominal LA than those with AIH and PBC, and patients with AILD complicated by LA may have marked cholestasis and liver impairment and poor treatment response, suggesting that LA might be used to determine disease severity and judge prognosis.
【关键字】:肝炎,自身免疫性; 胆管炎, 胆汁性; 重叠综合征; 淋巴系统疾病; 临床特征
【Key words】:hepatitis, autoimmune; cholangitis, biliary; AIH-PBC overlap syndrome; lymphatic diseases; clinical feature
【引证本文】:张璐,刘丽娜. 自身免疫性肝病并发腹腔淋巴结肿大的临床特征分析[J]. 临床肝胆病杂志, 2017, 33(11): 2173-2178.

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