Background 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. Methods 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. Findings 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. Interpretation 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. Data are available on reasonable request.

中文翻译：

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成人超短型乳糜泻的新实体：第一个国际队列和病例对照研究

背景 超短型乳糜泻 (USCD) 被定义为仅存在于十二指肠球部 (D1) 的绒毛萎缩，同时乳糜泻血清学呈阳性。我们提出了第一个针对 USCD 患者的多中心国际研究。方法 从 10 家三级医院（6 名来自欧洲、2 名来自亚洲、1 名来自北美和 1 名澳大利亚）确定 USCD 患者，并与年龄匹配和性别匹配的传统乳糜泻患者进行比较。结果 USCD 患者（n=137，中位年龄 27 岁，IQR 21-43 岁；73% 女性）比传统乳糜泻患者年轻（分别为 27 岁和 38 岁，p<0.001）。与传统乳糜泻患者相比，USCD 患者胃镜检查时的免疫球蛋白 A 组织转谷氨酰胺酶 (IgA-tTG) 滴度较低（1.8×正常上限 (ULN) (IQR 1.1–5.9) vs 12.6×ULN (IQR 3.3–18.3) ，p<0.001）。USCD 患者总体症状数量相同（中位数 3 (IQR 2-4) 与 3 (IQR 1-4)，p=0.875）。USCD 患者缺铁较少（41.8% vs 22.4%，p=0.006）。USCD 和传统乳糜泻均具有相同的上皮内淋巴细胞免疫表型染色模式；CD3 和 CD8 呈阳性，但 CD4 不呈阳性。在开始无麸质饮食 (GFD)（中位数 1181 天 IQR：440-2160 天）的随访中，USCD 以及年龄匹配和性别匹配的对照者的 IgA-tTG 滴度均出现类似的下降（0.5 ULN） (IQR 0.2–1.4) 与 0.7 ULN (IQR 0.2–2.6)，p=0.312）。95.7% 的 USCD 患者报告其症状有临床改善。解释 USCD 患者更年轻，有相似的症状负担，并且可以从 GFD 中受益。本研究赞同将 D1 取样作为内镜乳糜泻诊断检查一部分的建议。可根据合理要求提供数据。