New entity of adult ultra-short coeliac disease: the first international cohort and case-control study.
Study Goal
The researchers aimed to compare the clinical characteristics and response to a gluten-free diet (GFD) between patients with ultra-short coeliac disease (USCD) and conventional coeliac disease.
Results Summary
Patients with USCD showed similar symptomatic burden and clinical improvement (95.7%) on a GFD compared to conventional coeliac disease, with comparable reductions in IgA-tTG titres. The study supports GFD efficacy for USCD and recommends duodenal bulb sampling in diagnostic workups.
Population
Patients with USCD (n=137, median age 27, 73% female) and age/sex-matched conventional coeliac disease patients from 10 tertiary hospitals across multiple continents.
Effective Dosage
Not specified
Duration
Median follow-up of 1181 days (IQR: 440-2160 days)
Interactions
None mentioned
| Intervention | Direction | Endpoint | Population | Dosage | Impact | Claim # |
|---|---|---|---|---|---|---|
- | decrease | age | patients with USCD | 27 vs 38 years | were younger | #1 |
- | decrease | Immunoglobulin A-tissue transglutaminase (IgA-tTG) titres at index gastroscopy | 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) | were lower | #2 |
- | no change | symptoms overall | patients with USCD | median 3 (IQR 2-4) vs 3 (IQR 1-4) | had the same number | #3 |
- | decrease | iron deficiency | patients with USCD | 41.8% vs 22.4% | experienced less | #4 |
- | no change | intraepithelial lymphocytes immunophenotype staining pattern | both USCD and conventional coeliac disease | positive for CD3 and CD8, but not CD4 | had the same | #5 |
gluten-free diet (GFD) | decrease | IgA-tTG titres | both USCD and the age-matched and sex-matched controls | 0.5 ULN (IQR 0.2-1.4) vs 0.7 ULN (IQR 0.2-2.6) | experienced a similar reduction | #6 |
gluten-free diet (GFD) | decrease | symptoms | patients with USCD | 95.7% | reported a clinical improvement | #7 |
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.