Academic Unit of Gastroenterology, Sheffield Teaching Hospitals NHS Foundation Trust, Sheffield, UK
Division of Clinical Medicine, Faculty of Medicine and Population Health, The University of Sheffield Medical School, Sheffield, UK.
Gut. 2024 Jun 6;73(7):1124-1130. doi: 10.1136/gutjnl-2023-330913.
Ultra-short coeliac disease (USCD) is defined as villous atrophy only present in the duodenal bulb (D1) with concurrent positive coeliac serology. We present the first, multicentre, international study of patients with USCD.
Patients with USCD were identified from 10 tertiary hospitals (6 from Europe, 2 from Asia, 1 from North America and 1 from Australasia) and compared with age-matched and sex-matched patients with conventional coeliac disease.
Patients with USCD (n=137, median age 27 years, IQR 21-43 years; 73% female) were younger than those with conventional coeliac disease (27 vs 38 years, respectively, p<0.001). Immunoglobulin A-tissue transglutaminase (IgA-tTG) titres at index gastroscopy were lower in patients with USCD versus conventional coeliac disease (1.8×upper limit of normal (ULN) (IQR 1.1-5.9) vs 12.6×ULN (IQR 3.3-18.3), p<0.001).Patients with USCD had the same number of symptoms overall (median 3 (IQR 2-4) vs 3 (IQR 1-4), p=0.875). Patients with USCD experienced less iron deficiency (41.8% vs 22.4%, p=0.006).Both USCD and conventional coeliac disease had the same intraepithelial lymphocytes immunophenotype staining pattern; positive for CD3 and CD8, but not CD4.At follow-up having commenced a gluten-free diet (GFD) (median of 1181 days IQR: 440-2160 days) both USCD and the age-matched and sex-matched controls experienced a similar reduction in IgA-tTG titres (0.5 ULN (IQR 0.2-1.4) vs 0.7 ULN (IQR 0.2-2.6), p=0.312). 95.7% of patients with USCD reported a clinical improvement in their symptoms.
Patients with USCD are younger, have a similar symptomatic burden and benefit from a GFD. This study endorses the recommendation of D1 sampling as part of the endoscopic coeliac disease diagnostic workup.
超短型乳糜泻（USCD）被定义为仅在十二指肠球部（D1）存在绒毛萎缩，同时伴有阳性乳糜泻血清学。我们报告了首例多中心、国际性的 USCD 患者研究。
从 10 家三级医院（欧洲 6 家、亚洲 2 家、北美 1 家、澳大拉西亚 1 家）中确定 USCD 患者，并与年龄和性别匹配的常规乳糜泻患者进行比较。
USCD 患者（n=137，中位年龄 27 岁，IQR 21-43 岁；73%为女性）比常规乳糜泻患者年轻（分别为 27 岁和 38 岁，p<0.001）。与常规乳糜泻相比，USCD 患者的 IgA-组织转谷氨酰胺酶（IgA-tTG）在索引胃镜检查时的滴度较低（1.8×正常值上限（ULN）（IQR 1.1-5.9）比 12.6×ULN（IQR 3.3-18.3），p<0.001）。总体而言，USCD 患者的症状数量相同（中位数 3（IQR 2-4）比 3（IQR 1-4），p=0.875）。USCD 患者铁缺乏的发生率较低（41.8%比 22.4%，p=0.006）。USCD 和常规乳糜泻的上皮内淋巴细胞免疫表型染色模式相同；均为 CD3 和 CD8 阳性，但 CD4 阴性。在开始无麸质饮食（GFD）（中位数为 1181 天，IQR：440-2160 天）后，USCD 和年龄及性别匹配的对照组的 IgA-tTG 滴度均相似下降（0.5 ULN（IQR 0.2-1.4）比 0.7 ULN（IQR 0.2-2.6），p=0.312）。95.7%的 USCD 患者报告症状得到改善。
USCD 患者更年轻，具有相似的症状负担，并从 GFD 中受益。本研究支持将 D1 取样作为内镜乳糜泻诊断工作流程的一部分的建议。
