Ultra-short Celiac Disease Is a Distinct and Milder Phenotype of the Disease in Children

Background and Aims: Approximately 10% of children with celiac disease (CD) have ultra-short celiac disease (USCD), where histological abnormalities are limited to the duodenal bulb. The aim of our retrospective study was to identify clinical and serological characteristics at baseline and at follow-up of children with USCD. Methods: All children that were diagnosed with CD in our unit during 7/2010–12/2017, in whom biopsies were taken from duodenal bulb and second part, were included. We compared disease characteristics and course between children with USCD and children with involvement in the second part of the duodenum. Results: Out of 3740 children who underwent upper gastrointestinal endoscopies, 648 were diagnosed with CD. Seventy-one (11%) of those children had limited involvement in the duodenal bulb. The USCD group included more females (P = 0.021), were older (P = 0.005), had a lower prevalence of diarrhea (P = 0.003), anemia (P = 0.007), anti-tissue transglutaminase (TTG) antibodies count (P < 0.001) at presentation, lower frequency of endoscopic abnormality, lower Marsh score, and a trend toward shorter time to the normalization of anti-TTG antibodies under a gluten-free diet compared to the extensive CD. There were no differences in body mass index or duration of symptoms before diagnosis. Conclusion: Children with USCD presented with a distinct phenotype of milder symptoms, lower celiac serology, and milder endoscopic and histological findings, with a trend toward faster normalization under a gluten-free diet compared to those with extensive CD. Further studies are needed to determine the long-term course and prognosis of USCD.