Clinical and Immunologic Features of Ultra-short Celiac Disease

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Mooney, P. ; Kurien, M. ; Evans, K. ; Rosario, E. ; Cross, S. ; Vergani, P. ; Hadjivassiliou, M. ; Murray, J. ; Sanders, D. (2016)
  • Publisher: WB Saunders
  • Subject:
    mesheuropmc: nutritional and metabolic diseases

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