Department of Gastroenterology, Hospital Universitario de Caceres, Caceres, Spain.
Department of Gastroenterology, Hospital Universitario de Caceres, Caceres, Spain.
Gastroenterol Hepatol. 2022 Nov;45(9):652-659. doi: 10.1016/j.gastrohep.2022.03.011. Epub 2022 Apr 28.
Ultra-short coeliac disease (USCD) is a novel celiac disease (CD) subtype limited to the duodenal bulb (D1). HLA haplotypes and flow cytometry have not been assessed yet.
To compare genetic, clinical, serologic, histopathologic and inmmunophenotypic parameters between USCD and conventional celiac disease (CCD) patients.
Prospective single-center study in children and adult patients undergoing duodenal biopsies on a gluten-containing diet. Biopsies for histology and flow cytometry were taken separately from D1 and distal duodenum. Biopsies in seronegative patients with celiac lymphogram were repeated after 2 years on a gluten-free diet.
Among 505 included patients, 127 were diagnosed with CD, of whom 7 (5.5%) showed USCD. HLADQ2 was significantly less common in USCD compared to CCD (71% vs. 95%, p 0.003). Likewise, USCD patients showed more frequent non-significant seronegativity (28% vs. 8%, p 0.07) and significantly lower titrations (7-15IU/ml) of tissue transglutaminase antibodies (tTG-IgA) (60% vs. 13%, p<0.001). Biopsies from D1 revealed significant less NK cells down-expression in USCD patients (1.4 vs. 5, p 0.04).
Up to 5.5% of CD patients showed USCD. A lower frequency of HLADQ2, along with less serum tTG-IgA titration and duodenal NK cell suppression, were differential features of USCD.
超短型乳糜泻（USCD）是一种新型乳糜泻（CD）亚型，仅限于十二指肠球部（D1）。HLA 单倍型和流式细胞术尚未得到评估。
比较 USCD 和常规乳糜泻（CCD）患者的遗传、临床、血清学、组织病理学和免疫表型参数。
前瞻性单中心研究，纳入在含麸质饮食下接受十二指肠活检的儿童和成人患者。分别从 D1 和远端十二指肠采集组织学和流式细胞术活检。对血清学阴性且有乳糜泻淋巴造影的患者，在无麸质饮食 2 年后重复进行活检。
在 505 例纳入患者中，127 例诊断为 CD，其中 7 例（5.5%）为 USCD。与 CCD 相比，USCD 中 HLADQ2 明显较少见（71%比 95%，p<0.003）。同样，USCD 患者更频繁地出现非显著的血清阴性（28%比 8%，p=0.07）和显著较低的组织转谷氨酰胺酶抗体（tTG-IgA）滴度（7-15IU/ml）（60%比 13%，p<0.001）。从 D1 采集的活检显示，USCD 患者的 NK 细胞下调表达显著减少（1.4 比 5，p=0.04）。
高达 5.5%的 CD 患者表现为 USCD。HLADQ2 频率较低，以及血清 tTG-IgA 滴度较低和十二指肠 NK 细胞抑制，是 USCD 的不同特征。
