Article Text
Abstract
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.
- coeliac disease
- gluten free diet
- gluten sensitive enteropathy
- gluten
- celiac disease
Data availability statement
Data are available on reasonable request.
Statistics from Altmetric.com
Data availability statement
Data are available on reasonable request.
Footnotes
Twitter @DrSunnyR, @Mo_Shiha
Contributors SAR, EAG and DSS conceptualised and designed the study with comments from AS, GA, NV, CLAJ, CG, MM, OG, FJB-H, MGH, HAP, SSC, CC, KR, SA, AM, MR-N, FB, UV, MF, BL, PHRG, SL, JM-I, PMR, VV, LE, IS and AE. Data were collected by all authors and collated by SAR. Data analysis was completed by SAR and interpreted by all authors. Writing of the manuscript was completed by SAR and edited initially by DSS and then all authors. DSS is the guarantor. The final version was approved by all authors.
Funding DSS has previously received an educational grant from Dr Schaer (a gluten‐free food manufacturer). Dr Schaer has no involvement in this study. HAP funded by a Clinical Lecturers grant (CL-2021-04-002) from the NIHR.
Competing interests None declared.
Patient and public involvement Patients and/or the public were not involved in the design, or conduct, or reporting, or dissemination plans of this research.
Provenance and peer review Not commissioned; externally peer reviewed.