The Significance of Duodenal Mucosal Atrophy in Patients With Common Variable Immunodeficiency A Clinical and Histopathologic Study

The Significance of Duodenal Mucosal Atrophy in Patients With Common Variable Immunodeficiency A Clinical and Histopathologic Study
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DOI:
10.1309/ajcpeiilh2c0wfye
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发表时间:
2012-08-01
影响因子:
3.5
通讯作者:
Corazza, Gino R.
Corazza, Gino R.
中科院分区:
医学4区
文献类型:
--
作者:
Biagi, Federico;Bianchi, Paola I.;Corazza, Gino R.

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常见变异型免疫缺陷(CVID)患者可出现胃肠道表现和绒毛萎缩。在某些患者中,感染因子可能是原因,而在其他患者中,乳糜泻(CD)可能是原因。在这项研究中,我们调查的原因和组织病理学特征的患者与CVID。11例CVID和绒毛萎缩的患者进行了十二指肠活检,人类白细胞抗原(HLA)分型,并检测所有腹腔抗体。对照组为15例CVID患者和6例CD患者。对谷蛋白五饮食(GFD)的组织学反应允许11例患者中的3例诊断为CD。在其余8例中,对GFD或HLA分型缺乏组织学应答排除了CD。腹腔抗体给出了相互矛盾的结果,没有帮助。多形性浸润和病变,如移植物抗宿主病更常见于对GFD无反应的平坦粘膜。然而,这些发现的特异性仍有待确定,对GFD的反应仍然是这些患者CD的唯一诊断标准。11例CVID患者中有3例绒毛萎缩对谷蛋白敏感。在大多数患者中,这与谷蛋白反应性CD无关。
Gastrointestinal manifestations and villous atrophy can be seen in patients with common variable immunodeficiency (CVID). In some patients, infectious agents may be responsible, whereas in others, celiac disease (CD) may be the cause. In this study, we investigate the causes and the histopathologic features seen in patients with CVID. Eleven patients with CVID and villous atrophy underwent duodenal biopsies, human leukocyte antigen (HLA) typing, and testing for all celiac antibodies. Fifteen patients with CVID and normal villi and 6 patients with CD but without CVID served as controls. Histologic response to a gluten-five diet (GFD) allowed a diagnosis of CD in 3 of 11 patients. In the remaining 8, the lack of a histologic response to a GFD or HLA typing excluded CD. Celiac antibodies gave conflicting results and were of no help. Polymorphonuclear infiltrates and lesions like graft-versus-host disease are seen more often in flat mucosa unresponsive to a GFD. However, the specificity of these findings remains to be determined and response to a GFD remains the only diagnostic criteria for CD in these patients. Villous atrophy was gluten-sensitive in 3 of 11 patients with CVID. It was not related to gluten-responsive CD in most patients.