High prevalence of eosinophilic esophagitis in patients with inherited connective tissue disorders
High prevalence of eosinophilic esophagitis in patients with inherited connective tissue disorders
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DOI：
10.1016/j.jaci.2013.02.030
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发表时间：
2013-08-01
影响因子：
14.2
通讯作者：
Rothenberg, Marc E.
中科院分区：
文献类型：
--
作者：
Abonia, J. Pablo;Wen, Ting;Rothenberg, Marc E.
背景资料：嗜酸性粒细胞性食管炎（EoE）是一种新出现的慢性炎症性疾病，由多种食物引起的免疫超敏反应介导，与特应性和食管重塑密切相关。目的：我们提供临床和分子证据表明EoE在遗传性结缔组织病（CTD）患者中的高患病率。我们检查了CTD患者中Eoe的发生率，随后分析了有或无CTD特征的Eoe患者的食管mRNA转录谱。结果：我们报告了42例CTD样综合征EoE患者的队列，分别占我们医院范围内电子病历数据库和EoE研究注册表中CTD患者的0.8%和EoE患者的1.3%。CTD患者发生EoE的风险是普通人群的8倍（相对风险为8.1; 95%可信限为5.1-12.9;卡方2 = 5112.0; P < 10（-3））。食管转录谱确定了一个独特的基因子集，包括COL 8A 2，在患者EoE和CTDs.Conclusion：有一个显着的关联EoE与CTDs和证据的差异表达的基因参与结缔组织修复在这个队列。因此，我们建议将患有EoE和CTD的患者分层为称为EoE-CTD的子集。
Background: Eosinophilic esophagitis (EoE) is an emerging chronic inflammatory disease mediated by immune hypersensitization to multiple foods and strongly associated with atopy and esophageal remodeling.Objective: We provide clinical and molecular evidence indicating a high prevalence of EoE in patients with inherited connective tissue disorders (CTDs).Methods: We examined the rate of EoE among patients with CTDs and subsequently analyzed esophageal mRNA transcript profiles in patients with EoE with or without CTD features.Results: We report a cohort of 42 patients with EoE with a CTD-like syndrome, representing 0.8% of patients with CTDs and 1.3% of patients with EoE within our hospital-wide electronic medical record database and our EoE research registry, respectively. An 8-fold risk of EoE in patients with CTDs (relative risk, 8.1; 95% confidence limit, 5.1-12.9; chi(2) 1=5 112.0; P < 10(-3)) was present compared with the general population. Esophageal transcript profiling identified a distinct subset of genes, including COL8A2, in patients with EoE and CTDs.Conclusion: There is a remarkable association of EoE with CTDs and evidence for a differential expression of genes involved in connective tissue repair in this cohort. Thus, we propose stratification of patients with EoE and CTDs into a subset referred to as EoE-CTD.