Does Short Segment Celiac Disease Confined to the Duodenal Bulb Have a Unique Clinical, Serological or Histological Profile?

Objectives: Celiac disease (CD) is a common autoimmune enteropathy with increased prevalence worldwide. The diagnosis is based on positive serological tests with proved histological findings. Due to possible patchy villous atrophy pattern, the histological features may be present only at the duodenal bulb in about 11% of patients. So far, any possible association between CD confined to the duodenal bulb, and a unique clinical profile has not been fully explored.
We aimed to investigate whether short segment Celiac disease (SSCD) in pediatric patients has a unique demographic, clinical, laboratory or histological profile at the time of diagnosis compared to extensive CD.

Methods: A retrospective study including all children, 1-18 years who underwent upper endoscopy with duodenal biopsies for a suspected CD at our Pediatric Gastroenterology Unit between 01/2013 and 08/2108. The diagnosis of CD was made in accordance with the ESPGHAN criteria. Demographic, clinical data, family history of CD, laboratory results at diagnosis and histological data were recorded. Histological severity was assessed by MARSH classification.

Results: Out of 160 suspected CD patients, 22 and 19 children were excluded due to lack of data. 22 of the remaining 113 children had SSCD (19%). The only difference between SSCD and more extensive CD was that children diagnosed with SSCD had lower level of anti-TTG antibodies at presentation (p< 0.01).
No significant differences were documented regarding demographic, clinical and histological features.

Conclusions: SSCD is more common than previously reported. Despite lower serology titer the histological severity is similar.