J Pediatr Gastroenterol Nutr. 2022 Sep 19. doi: 10.1097/MPG.0000000000003613.Online ahead of print.

Telly Cheung ¹, Edwin F de Zoeten ¹, Edward J Hoffenberg ¹, Edwin Liu ¹, Zhaoxing Pan ², Thomas Walker ¹, Marisa Stahl ¹

Author information

¹Digestive Health Institute, Department of Pediatrics, School of Medicine, University of Colorado Anschutz Medical Campus, Aurora, CO, United States.

²Biostatistics Core of Children's Hospital Colorado Research Institute, School of Medicine, University of Colorado Anschutz Medical Campus, Aurora, CO, United States.

Abstract

Objectives: Celiac disease (CeD) autoimmunity and coexisting inflammatory bowel disease (IBD) present a diagnostic dilemma. Our aims were to describe the phenotype of children with IBD and CeD seropositivity and evaluate provider confidence for diagnosing CeD in this population.

Methods: We performed a single-center retrospective cohort study of subjects ≤ 18 years old with IBD and CeD seropositivity between 2006-2020. Subjects were considered to have IBD-CeD if they met CeD diagnosis by serology and histology per NASPGHAN guidelines and if providers suspected CeD as evaluated by a survey. The IBD-only cohort included seropositive participants that did not meet criteria for CeD. Demographic, histologic, gross endoscopic, and laboratory features were compared using Fisher's exact test.

Results: Of 475 children with IBD, eight had concomitant CeD: five had tissue transglutaminase (tTG) IgA > 10x ULN (p = 0.006) and eight had villous atrophy (VA, p = 0.003) when compared with 17 seropositive participants with IBD-only. No children with IBD-CeD had esophageal eosinophilia, duodenal cryptitis, duodenal ulceration, or fecal calprotectin > 250 ug/g. Factors that contributed to provider uncertainty for diagnosing CeD in IBD included: the absence of villous atrophy and intraepithelial lymphocytes, the presence of neutrophilic and eosinophilic duodenitis, diffuse ulceration, elevated inflammatory markers, and immunosuppression therapy.

Conclusions: Diagnosing CeD in children with IBD continues to be challenging. Although high titers of tTG IgA and VA increased provider confidence for diagnosing CeD in IBD, development of evidence-based guidelines are needed. They should better assess the importance of features atypical of concomitant CeD that contribute to uncertainty.