Oral Presentation International Celiac Disease Symposium 2026

A dual role for IFNγ in epithelial injury and crypt hyperplasia in celiac disease (146139)

Joram Mooiweer 1 , Sajid Anwar 1 , Nathan V Ribeiro 1 , Aarón D Ramírez-Sánchez 2 , Gieneke Gonera 3 , Margreet Wessels 4 , Cisca Wijmenga 1 , Sebo Withoff 1 , Iris H Jonkers 1
  1. Department of Genetics, University Medical Center Groningen, Groningen, The Netherlands
  2. Department of Genetics, Physical Anthropology and Animal Physiology, University of the Basque Country UPV/EHU, Leioa, Basque, Spain
  3. Department of Pediatrics, Wilhelmina Children's Hospital, Assen, Drenthe, The Netherlands
  4. Department of Pediatrics, Rijnstate Hospital, Arnhem, Gelderland, The Netherlands

Celiac disease (CeD) is characterized by villous atrophy, crypt hyperplasia, and chronic intestinal inflammation. Gluten-driven immune activation ultimately leads to the activation of CD8⁺ intraepithelial lymphocytes (IELs), which target the epithelium and contribute to villous atrophy. However, the precise immune–epithelial mechanisms underlying epithelial remodeling remain incompletely understood. To address this, we combined single-cell RNA sequencing of duodenal biopsies with an autologous organoid–IEL co-culture model established from matched patient biopsies.

Single-cell analysis of CeD biopsies revealed marked epithelial remodeling, with loss of mature enterocytes and expansion of transit-amplifying (TA) cells with a pronounced IFNγ-response signature. These TA cells were predicted to interact with IELs, suggesting a link between IEL activity and epithelial stress remodeling. To test this functionally, we generated patient-derived intestinal organoids and matched CD8αβ⁺TCRαβ⁺ IEL lines and studied their interactions in co-culture. Upon IL-15 and IL-21 stimulation, CeD-derived co-cultures showed increased epithelial cell death together with elevated IFNγ secretion. TA-rich epithelial organoids presented a strong IFNγ-associated transcriptional program, recapitulating the epithelial state observed in CeD biopsies. IFNGR1 blockade or genetic knockout in organoids reduced IEL-mediated epithelial cell death, supporting a contribution of epithelial IFNγ signaling to the damage phenotype. Direct IFNγ exposure induced a similar IFNγ program in organoids, but did not increase apoptosis. Instead, prolonged IFNγ stimulation increased organoid growth, consistent with crypt hyperplasia. Accordingly, IFNGR1-deficient organoids were protected from the IFNγ-induced increase in growth.

Together, these findings identify IFNγ as a central mediator of CeD epithelial pathology with a dual role: promoting epithelial injury in the context of IEL activation, while driving epithelial proliferation and crypt hyperplasia upon sustained exposure. The autologous organoid–IEL co-culture model recapitulates key features of CeD and provides a platform to study immune-driven epithelial remodeling and therapeutic targeting of IFNγ signaling.