A Cross-Sectional Study of the Prevalence of Gastrointestinal Symptoms and Pathology in Patients With Common Variable Immunodeficiency
A Cross-Sectional Study of the Prevalence of Gastrointestinal Symptoms and Pathology in Patients With Common Variable Immunodeficiency
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DOI：
10.1038/ajg.2016.329
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发表时间：
2016-10-01
影响因子：
9.8
通讯作者：
Fevang, Borre
中科院分区：
文献类型：
--
作者：
Jorgensen, Silje F.;Reims, Henrik M.;Fevang, Borre
目的：本研究的目的是研究常见变异型免疫缺陷（CVID）患者的胃肠道（GI）症状和组织病理学的患病率，并将结果与GI感染和全身免疫activation.METHODS标志物联系起来：在这项横断面研究中，我们讨论了103例患者的GI症状和53例接受上下内镜检查的患者的GI组织病理学结果。最常见的组织病理学结果与GI症状、B细胞表型和全身免疫激活标志物（可溶性CD 14、sCD 25和sCD 163）相关。微阵列分析比较了CVID中的“乳糜泻样疾病”与乳糜泻。筛选选定的细菌和病毒感染的粪便样本和肠道粘膜活检performed.RESULTS：本研究的主要结果如下：最常见的胃肠道症状腹胀（34%），疼痛（30%），腹泻（26%）。最常见的组织病理学发现是十二指肠降部上皮内淋巴细胞增加，即，“乳糜泻样疾病”（46%的患者）、胃肠道粘膜浆细胞数量减少（62%）和淋巴增生（38%），这些均与胃肠道症状无关。GI粘膜浆细胞减少与CVID的B细胞表型特征相关，血清sCD 14（P=0.025）、sCD 25（P=0.01）和sCD 163（P=0.04）水平升高。微阵列分析区分CVID患者与“乳糜泻样疾病”和乳糜泻。粪便样本和肠道粘膜活检中细菌和病毒感染的阳性检测很少，包括活检标本中诺如病毒的PCR检测（0阳性检测）。结论：总之，胃肠道病理学在CVID中很常见，但不一定会引起症状。然而，GI粘膜中浆细胞减少与全身免疫激活有关，CVID中的“乳糜泻样疾病”和真正的乳糜泻似乎是不同的疾病实体，如通过基因表达评估的，并且感染（包括诺如病毒）很少是CVID肠病的原因。
OBJECTIVES: The objective of this study was to study the prevalence of gastrointestinal (GI) symptoms and histopathology in patients with common variable immunodeficiency (CVID) as well as linking the findings to GI infections and markers of systemic immune activation.METHODS: In this cross-sectional study, we addressed GI symptoms in 103 patients and GI histopathological findings in 53 patients who underwent upper and lower endoscopic examination. The most frequent histopathological findings were linked to GI symptoms, B-cell phenotype, and markers of systemic immune activation (soluble (s) CD14, sCD25, and sCD163). Microarray analysis compared "celiac-like disease" in CVID to celiac disease. Screening for selected bacterial and viral infections in fecal samples and gut mucosal biopsies was performed.RESULTS: The main findings of this study were as follows: most common GI symptoms were bloating (34%), pain (30%), and diarrhea (26%). The most frequent histopathological findings were increased intraepithelial lymphocytes in the descending part of the duodenum, i.e., "celiac-like disease" (46% of patients), decreased numbers of plasma cells in GI tract mucosa (62%), and lymphoid hyperplasia (38%), none of which were associated with GI symptoms. Reduced plasma cells in GI mucosa were associated with B-cell phenotypic characteristics of CVID, and increased serum levels of sCD14 (P=0.025), sCD25 (P=0.01), and sCD163 (P=0.04). Microarray analyses distinguished between CVID patients with "celiac-like disease" and celiac disease. Positive tests for bacterial and viral infections were scarce both in fecal samples and gut mucosal biopsies, including PCR test for norovirus in biopsy specimens (0 positive tests).CONCLUSIONS: In conclusion, GI pathology is common in CVID, but does not necessarily cause symptoms. However, reduced plasma cells in GI mucosa were linked to systemic immune activation, "celiac-like disease" in CVID and true celiac disease appear to be different disease entities, as assessed by gene expression, and infections (including norovirus) are rarely a cause of the CVID enteropathy.