Celiac.com 04/06/2016 - Ultra-short celiac disease (USCD) is a type of celiac disease in which villous atrophy limited to the patient's duodenal bulb.

Photo: CC--GeorgeThe clinical effects of of ultra-short celiac disease, or gluten-sensitive enteropathy with villous atrophy limited to the duodenal bulb (D1) have not been delineated in adults with celiac disease.

A team of researchers recently evaluated the sensitivity of D1 biopsy analysis in celiac disease detection, the number and sites of biopsies required to detect USCD, which is villous atrophy limited to the duodenal bulb, and the clinical characteristics of USCD.

The researchers included Peter D. Mooney, Matthew Kurien, Kate E. Evans, Eleanor Rosario, Simon S. Cross, Patricia Vergani, Marios Hadjivassiliou, Joseph A. Murray, and David S. Sanders. They are variously affiliated with the University of Sheffield, UK, and with the Departments of Gastroenterology, Neurology and Histopathology at Royal Hallamshire Hospital, Sheffield, UK.

For their evaluation, they conducted a prospective study of 854 women and 524 men, averaging about 50 years of age, who underwent endoscopy at a tertiary medical center in the United Kingdom from 2008 through 2014. The team collected routine duodenal biopsies from D1 and D2.

They collected quadrantic D1 biopsies from 171 consecutive patients with a high suspicion of celiac disease. This group averaged about age 46 years of age, and was 64% female.

Using biopsy analysis, they then compared the clinical data from patients diagnosed with USCD against data from patients with conventional celiac disease damage beyond D1, and against data from a control group of patients without celiac disease. They then compared numbers of intraepithelial lymphocytes (IELs) and immune phenotypes between D1 vs D2 in patients with celiac disease.

Of the 1378 patients assessed, they diagnosed 268, nearly 20%, with celiac disease; 9.7% of these patients had villous atrophy confined to D1 (USCD, P<.0001). By collecting just a single additional biopsy from any D1 site, the sensitivity of celiac disease detection increased by about 10% (P<.0001).

Overall, patients with USCD were younger, had lower levels of tissue transglutaminase antibody, and suffered less frequently with diarrhea than patients with conventional celiac disease. Both USCD and control patients had far fewer cases ferritin deficiency or folate deficiency than patients with conventional celiac disease. Patients with celiac disease averaged 50 IELs/100 enterocytes in D1, and 48 IELs/100 enterocytes in D2.

The phenotype of IELs from patients with D1 celiac disease was no different from those of patients with D2 celiac disease.

This study shows that the collection of a single additional biopsy from any site in the D1 intestine increases the sensitivity of detection for celiac disease by about 10%.

Patients with USCD may have early-stage or limited celiac disease, with a mild clinical phenotype, and fewer nutritional deficiencies. This is an important thing to keep in mind when diagnosing and treating such cases.

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