title: Occurrence of large granular lymphocytes and natural killer cells in the epithelium of the gut distinguishes two different coeliac diseases.
abstract: In a longterm study, we have divided coeliac disease into two distinct entities (abortive and permanent) based on the occurrence of large granular lymphocytes and natural killer cells within the epithelium of the gut. The natural killer and large granular lymphocytes cells were characterised by either immunohistochemical or phase contrast microscopical procedures on the initial biopsies from 15 children with coeliac disease. They were compared with seven individuals with partial villus atrophy and eight with normal villous morphology. Although the histological findings were similar in the initial biopsies of all patients with coeliac disease, the patients with permanent coeliac disease had a significantly lower number (0.41(0.61)cells/mm2) of large granular lymphocytes and natural killer cells compared with those patients with abortive coeliac disease (11.93 (6.23) cells/mm2). Those in the permanent group developed a significantly more pronounced flat mucosa after gluten challenge or provocation compared with the abortive group and had to remain on a strict gluten free diet in contrast with those in the abortive group. Thus, the occurrence of intraepithelial large granular lymphocytes and natural killer cells characterises two distinctly different coeliac diseases. Based on our results neither the histological evaluation of the biopsy nor the utilisation of the revised European Society for Paediatric Gastroenterology and Nutrition (ESPGAN) Criteria are adequate in diagnosing the two types of coeliac disease.
authors.affiliation: Department of Gastroenterology and Electron Microscopy, University Children's Hospital, Basel, Switzerland.
authors.forename: F
authors.initials: F
authors.lastname: Hadziselimovic
authors.forename: L R
authors.initials: LR
authors.lastname: Emmons
authors.forename: U
authors.initials: U
authors.lastname: Schaub
authors.forename: E
authors.initials: E
authors.lastname: Signer
authors.forename: A
authors.initials: A
authors.lastname: Bürgin-Wolff
authors.forename: R
authors.initials: R
authors.lastname: Krstic
mesh_terms: D002446:Celiac Disease / Q000178:diet therapy / Q000473:pathology*; D002675:Child, Preschool; D003594:Cytoplasmic Granules / Q000648:ultrastructure; D005500:Follow-Up Studies; D005983:Glutens / Q000008:administration & dosage; D006801:Humans; D007413:Intestinal Mucosa / Q000648:ultrastructure*; D007694:Killer Cells, Natural / Q000648:ultrastructure*; D008214:Lymphocytes / Q000648:ultrastructure*; D008854:Microscopy, Electron; D008855:Microscopy, Electron, Scanning
chemical_list: D005983:Glutens