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CVID 患者的肠道组织学显示出独特的黏膜炎症模式。

Bowel Histology of CVID Patients Reveals Distinct Patterns of Mucosal Inflammation.

机构信息

Institute of Immunity and Transplantation, Royal Free Hospital, University College London, London, UK.

Institute for Immunodeficiency, Center for Chronic Immunodeficiency, Center for Translational Cell Research, Medical Center, Faculty of Medicine, Albert-Ludwigs-University of Freiburg, Breisacher Str. 115, 79106, Freiburg, Germany.

出版信息

J Clin Immunol. 2022 Jan;42(1):46-59. doi: 10.1007/s10875-021-01104-5. Epub 2021 Oct 1.

Abstract

Diarrhea is the commonest gastrointestinal symptom in patients with common variable immunodeficiency (CVID). Different pathologies in patients' bowel biopsies have been described and links with infections have been demonstrated. The aim of this study was to analyze the bowel histology of CVID patients in the Royal-Free-Hospital (RFH) London CVID cohort. Ninety-five bowel histology samples from 44 adult CVID patients were reviewed and grouped by histological patterns. Reasons for endoscopy and possible causative infections were recorded. Lymphocyte phenotyping results were compared between patients with different histological features. There was no distinctive feature that occurred in most diarrhea patients. Out of 44 patients (95 biopsies), 38 lacked plasma cells. In 14 of 21 patients with nodular lymphoid hyperplasia (NLH), this was the only visible pathology. In two patients, an infection with Giardia lamblia was associated with NLH. An IBD-like picture was seen in two patients. A coeliac-like picture was found in six patients, four of these had norovirus. NLH as well as inflammation often occurred as single features. There was no difference in blood lymphocyte phenotyping results comparing groups of histological features. We suggest that bowel histology in CVID patients with abdominal symptoms falls into three major histological patterns: (i) a coeliac-like histology, (ii) IBD-like changes, and (iii) NLH. Most patients, but remarkably not all, lacked plasma cells. CVID patients with diarrhea may have an altered bowel histology due to poorly understood and likely diverse immune-mediated mechanisms, occasionally driven by infections.

摘要

腹泻是普通变异性免疫缺陷(CVID)患者最常见的胃肠道症状。患者肠道活检中已经描述了不同的病理学,并已证明与感染有关。本研究旨在分析伦敦皇家自由医院(RFH)CVID 队列中 CVID 患者的肠道组织学。对 44 例成年 CVID 患者的 95 例肠道组织学样本进行了回顾性分析,并按组织学模式进行分组。记录了内镜检查的原因和可能的致病感染。比较了不同组织学特征患者的淋巴细胞表型结果。大多数腹泻患者没有明显的特征。在 44 例患者(95 例活检)中,有 38 例缺乏浆细胞。在 21 例结节性淋巴组织增生(NLH)患者中,有 14 例患者仅有这种可见的病理学改变。在 2 例患者中,NLH 与蓝氏贾第鞭毛虫感染有关。2 例患者出现 IBD 样表现。6 例患者出现类乳糜泻表现,其中 4 例患者感染诺如病毒。NLH 和炎症通常作为单一特征出现。比较组织学特征组之间的血液淋巴细胞表型结果没有差异。我们建议,有腹部症状的 CVID 患者的肠道组织学可分为三种主要的组织学模式:(i)类乳糜泻组织学,(ii)IBD 样改变,和(iii)NLH。大多数患者(但并非所有患者)缺乏浆细胞。由于免疫介导的机制尚不清楚且可能多种多样,腹泻的 CVID 患者的肠道组织学可能发生改变,偶尔也可能由感染驱动。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a8ec/8821476/b60a520656b6/10875_2021_1104_Fig1_HTML.jpg

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