Abstract：
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.
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.
摘要：
背景：超短乳糜泻（USCD）定义为仅存在于十二指肠球部（D1）并伴有腹腔血清学阳性的绒毛萎缩。我们提出了第一个，多中心,USCD患者的国际研究。
方法：从10家三级医院（6家来自欧洲，2来自亚洲1名来自北美，1名来自澳大利亚），并与年龄匹配和性别匹配的常规乳糜泻患者进行比较。
结果：USCD患者（n=137，中位年龄27岁，IQR21-43岁；73%的女性）比传统乳糜泻患者年轻(27岁vs38岁，分别,p<0.001)。USCD患者在胃镜检查时的免疫球蛋白A-组织转谷氨酰胺酶（IgA-tTG）滴度低于常规乳糜泻（1.8×正常上限（ULN）（IQR1.1-5.9）vs12.6×ULN（IQR3.3-18.3），p<0.001)。USCD患者的症状总数相同（中位数3（IQR2-4）vs3（IQR1-4），p=0.875)。USCD患者铁缺乏较少（41.8%vs22.4%，p=0.006)。USCD和常规乳糜泻都具有相同的上皮内淋巴细胞免疫表型染色模式；CD3和CD8阳性，但CD4阳性。在开始无麸质饮食（GFD）（中位数为1181天，IQR：440-2160天）的随访中，USCD和年龄匹配和性别匹配的对照组的IgA-tTG滴度均有类似的降低（0.5ULN（IQR0.2-1.4）对0.7ULN（IQR0.2-2.6），p=0.312）。95.7%的USCD患者报告其症状有临床改善。
结论：USCD患者更年轻，有类似的症状负担和受益于GFD。这项研究支持建议将D1采样作为内窥镜乳糜泻诊断检查的一部分。
方法：从10家三级医院（6家来自欧洲，2来自亚洲1名来自北美，1名来自澳大利亚），并与年龄匹配和性别匹配的常规乳糜泻患者进行比较。
结果：USCD患者（n=137，中位年龄27岁，IQR21-43岁；73%的女性）比传统乳糜泻患者年轻(27岁vs38岁，分别,p<0.001)。USCD患者在胃镜检查时的免疫球蛋白A-组织转谷氨酰胺酶（IgA-tTG）滴度低于常规乳糜泻（1.8×正常上限（ULN）（IQR1.1-5.9）vs12.6×ULN（IQR3.3-18.3），p<0.001)。USCD患者的症状总数相同（中位数3（IQR2-4）vs3（IQR1-4），p=0.875)。USCD患者铁缺乏较少（41.8%vs22.4%，p=0.006)。USCD和常规乳糜泻都具有相同的上皮内淋巴细胞免疫表型染色模式；CD3和CD8阳性，但CD4阳性。在开始无麸质饮食（GFD）（中位数为1181天，IQR：440-2160天）的随访中，USCD和年龄匹配和性别匹配的对照组的IgA-tTG滴度均有类似的降低（0.5ULN（IQR0.2-1.4）对0.7ULN（IQR0.2-2.6），p=0.312）。95.7%的USCD患者报告其症状有临床改善。
结论：USCD患者更年轻，有类似的症状负担和受益于GFD。这项研究支持建议将D1采样作为内窥镜乳糜泻诊断检查的一部分。
