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十二指肠黏膜萎缩在普通变异性免疫缺陷患者中的意义:一项临床和组织病理学研究。

The significance of duodenal mucosal atrophy in patients with common variable immunodeficiency: a clinical and histopathologic study.

机构信息

Coeliac Centre/First Department of Internal Medicine, Fondazione IRCCS Policlinico San Matteo, Pavia, Italy.

出版信息

Am J Clin Pathol. 2012 Aug;138(2):185-9. doi: 10.1309/AJCPEIILH2C0WFYE.

DOI:10.1309/AJCPEIILH2C0WFYE
PMID:22904128
Abstract

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-free 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.

摘要

胃肠道表现和绒毛萎缩可见于普通变异性免疫缺陷病(CVID)患者。在某些患者中,感染因子可能是病因,而在其他患者中,乳糜泻(CD)可能是病因。在这项研究中,我们调查了 CVID 患者的病因和组织病理学特征。11 例 CVID 伴绒毛萎缩患者接受了十二指肠活检、人类白细胞抗原(HLA)分型以及所有乳糜泻抗体检测。15 例 CVID 伴正常绒毛和 6 例 CD 但无 CVID 的患者作为对照。无麸质饮食(GFD)的组织学反应允许在 11 例患者中的 3 例中诊断为 CD。在其余 8 例中,缺乏对 GFD 的组织学反应或 HLA 分型排除了 CD。乳糜泻抗体的结果相互矛盾,没有帮助。多形核细胞浸润和移植物抗宿主病样病变在无 GFD 反应的扁平黏膜中更常见。然而,这些发现的特异性仍有待确定,并且 GFD 仍然是这些患者 CD 的唯一诊断标准。在 11 例 CVID 患者中,有 3 例绒毛萎缩对麸质敏感。在大多数患者中,它与麸质反应性 CD 无关。

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