Common variable immunodeficiency (CVID), the most common symptomatic primary antibody deficiency, is accompanied in some patients by a duodenal inflammation and malabsorption syndrome known as CVID enteropathy (E-CVID).The goal of this study was to investigate the immunological abnormalities in CVID patients that lead to enteropathy as well as the contribution of intestinal microbiota to this process.We found that, in contrast to noE-CVID patients (without enteropathy), E-CVID patients have exceedingly low levels of IgA in duodenal tissues. In addition, using transkingdom network analysis of the duodenal microbiome, we identified Acinetobacter baumannii as a candidate pathobiont in E-CVID. Finally, we found that E-CVID patients exhibit a pronounced activation of immune genes and down-regulation of epithelial lipid metabolism genes. We conclude that in the virtual absence of mucosal IgA, pathobionts such as A. baumannii, may induce inflammation that re-directs intestinal molecular pathways from lipid metabolism to immune processes responsible for enteropathy.

常见变异型免疫缺陷 (CVID) 是最常见的有症状的一抗缺陷，部分患者伴有十二指肠炎症和吸收不良综合征，称为 CVID 肠病 (E-CVID)。本研究的目的是调查 CVID 中的免疫学异常我们发现，与 noE-CVID 患者（无肠病）相比，E-CVID 患者十二指肠组织中 IgA 水平极低。此外，通过对十二指肠微生物组的跨界网络分析，我们将鲍曼不动杆菌确定为 E-CVID 中的候选病原体。最后，我们发现 E-CVID 患者表现出免疫基因的显着激活和上皮脂质代谢基因的下调。我们得出的结论是，在粘膜 IgA 几乎不存在的情况下，鲍曼不动杆菌等病原体可能会诱发炎症，从而将肠道分子途径从脂质代谢重新引导至负责肠病的免疫过程。