57 research outputs found
Clinical and Immunologic Features of Ultra-short Celiac Disease
BACKGROUND & AIMS: The clinical effects of gluten-sensitive
enteropathy with villous atrophy limited to the duodenal bulb
(D1) have not been delineated in adults with celiac disease. We
investigated the sensitivity of D1 biopsy analysis in the detection
of celiac disease, the number and sites of biopsies required
to detect ultra-short celiac disease (USCD, villous atrophy
limited to D1), and the clinical phenotype of USCD. METHODS:
We performed a prospective study of 1378 patients (mean age,
50.3 y; 62% female) who underwent endoscopy at a tertiary
medical center in the United Kingdom from 2008 through
2014; routine duodenal biopsy specimens were collected from
D1 and the second part of the duodenum (D2). Quadrantic D1
biopsy specimens were collected from 171 consecutive patients
with a high suspicion of celiac disease (mean age, 46.5 y; 64%
female). Clinical data from patients diagnosed with USCD, based
on biopsy analysis, were compared with those from patients
with conventional celiac disease (CCD) (villous atrophy beyond
D1) and individuals without celiac disease (controls). The
number of intraepithelial lymphocytes (IELs) and immune
phenotypes were compared between D1 vs D2 in patients with
celiac disease. RESULTS: Of the 1378 patients assessed, 268
(19.4%) were diagnosed with celiac disease; 9.7% of these
patients had villous atrophy confined to D1 (USCD; P < .0001).
Collection of a single additional biopsy specimen from any D1
site increased the sensitivity of celiac disease detection by
9.3%–10.8% (P < .0001). Patients with USCD were younger
(P ¼ .03), had lower titers of tissue transglutaminase antibody
(P ¼ .001), and less frequently presented with diarrhea
(P ¼ .001) than patients with CCD. Higher proportions of
patients with CCD had ferritin deficiency (P ¼ .007) or folate
deficiency (P ¼ .003) than patients with USCD or controls.
Patients with celiac disease had a median of 50 IELs/100
enterocytes in D1 and a median of 48 IELs/100 enterocytes
(P ¼ .7) in D2. The phenotype of IELs from patients with D1
celiac disease was indistinguishable from those of patients with
D2 celiac disease. CONCLUSIONS: Collection of a single
additional biopsy specimen from any site in the D1 intestine
increases the sensitivity of detection for celiac disease. Patients
with USCD may have early stage or limited celiac disease, with a
mild clinical phenotype and infrequent nutritional deficiencies
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