原发性胆汁性肝硬化患者一级亲属中的原发性胆汁性肝硬化特异性自身抗体。

Primary biliary cirrhosis-specific autoantibodies in first degree relatives of Greek primary biliary cirrhosis patients.

机构信息

Department of Medicine and Research Laboratory of Internal Medicine, Medical School, University of Thessaly, 41110 Larissa, Greece.

出版信息

World J Gastroenterol. 2012 Sep 14;18(34):4721-8. doi: 10.3748/wjg.v18.i34.4721.

DOI:10.3748/wjg.v18.i34.4721

PMID:23002341

原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC3442210/

Abstract

AIM

To determine the prevalence and significance of primary biliary cirrhosis (PBC)-specific autoantibodies in first-degree relatives (FDRs) of Greek PBC patients.

METHODS

The presence of antimitochondrial antibodies (AMA) and PBC-specific antinuclear antibodies (ANA) were determined using indirect immunofluorescence assays, dot-blot assays, and molecularly based enzyme-linked immunosorbent assays in 101 asymptomatic for liver-related symptoms FDRs of 44 PBC patients. In order to specify our results, the same investigation was performed in 40 healthy controls and in a disease control group consisting of 40 asymptomatic for liver-related symptoms FDRs of patients with other autoimmune liver diseases namely, autoimmune hepatitis-1 or primary sclerosing cholangitis (AIH-1/PSC).

RESULTS

AMA positivity was observed in 19 (only 4 with abnormal liver function tests) FDRs of PBC patients and none of the healthy controls. The prevalence of AMA was significantly higher in FDRs of PBC patients than in AIH-1/PSC FDRs and healthy controls [18.8%, 95% confidence interval (CI): 12%-28.1% vs 2.5%, 95% CI: 0.1%-14.7%, P = 0.01; 18.8%, 95% CI: 12%-28.1% vs 0%, 95% CI: 0%-10.9%, P = 0.003, respectively]. PBC-specific ANA positivity was observed in only one FDR from a PSC patient. Multivariate analysis showed that having a proband with PBC independently associated with AMA positivity (odds ratio: 11.24, 95% CI: 1.27-25.34, P = 0.03) whereas among the investigated comorbidities and risk factors, a positive past history for urinary tract infections (UTI) was also independently associated with AMA detection in FDRs of PBC patients (odds ratio: 3.92, 95% CI: 1.25-12.35, P = 0.02).

CONCLUSION

In FDRs of Greek PBC patients, AMA prevalence is significantly increased and independently associated with past UTI. PBC-specific ANA were not detected in anyone of PBC FDRs.

摘要

目的

确定原发性胆汁性肝硬化（PBC）患者一级亲属（FDRs）中 PBC 特异性自身抗体的流行率和意义。

方法

采用间接免疫荧光法、斑点印迹法和基于分子的酶联免疫吸附试验，在 44 例 PBC 患者的 101 例无症状肝相关症状 FDRs 中检测抗线粒体抗体（AMA）和 PBC 特异性抗核抗体（ANA）。为了明确我们的研究结果，我们在 40 名健康对照者和 40 名无症状肝相关症状 FDRs 的疾病对照组中进行了相同的研究，这些对照组的疾病包括其他自身免疫性肝病，即自身免疫性肝炎 1 型或原发性硬化性胆管炎（AIH-1/PSC）。

结果

在 44 例 PBC 患者的 19 例（仅 19 例肝功能异常）FDRs 中观察到 AMA 阳性，而在健康对照组中均未观察到。与 AIH-1/PSC FDRs 和健康对照组相比，PBC FDRs 中 AMA 的阳性率明显更高[18.8%，95%置信区间（CI）：12%-28.1%比 2.5%，95%CI：0.1%-14.7%，P=0.01；18.8%，95%CI：12%-28.1%比 0%，95%CI：0%-10.9%，P=0.003]。仅在一名 PSC 患者的 FDR 中观察到 PBC 特异性 ANA 阳性。多变量分析显示，先证者患有 PBC 与 AMA 阳性独立相关（比值比：11.24，95%CI：1.27-25.34，P=0.03），而在研究的合并症和危险因素中，尿路感染（UTI）的阳性既往史也与 PBC FDRs 中 AMA 的检测独立相关（比值比：3.92，95%CI：1.25-12.35，P=0.02）。