title: T-lymphocyte dysfunctions occurring together with apical gut epithelial cell autoantibodies.
abstract: Gut epithelial cell autoantibodies have been considered a hallmark of autoimmune enteropathy, a disorder occurring in children with protracted diarrhea of unknown etiology. Four patients (two male and two female) with such autoantibodies were studied. Immunofluorescence analysis showed two different disjunctive staining patterns: complement-fixing apical (three of four) and cytoplasmic (the remaining fourth one), which are shown to be directed against different structures. All three patients positive for complement-fixing apical gut epithelial cell autoantibodies had abnormal T-cell responses in vitro, one of them with an immunoglobulin G2 immunoglobulin deficiency and another with an immunoglobulin A deficiency. An immunoglobulin A deficiency without T-cell alterations was also diagnosed in the cytoplasmic gut epithelial cell autoantibody-positive patient. These findings suggest that different immunologic alterations (either a T-cell abnormality or immunoglobulin deficiency) may favor the appearance of gut epithelial cell autoantibodies (complement-fixing apical or cytoplasmic, respectively). Furthermore, these autoantibodies should not be considered a specific marker of autoimmune enteropathy, because they may not always be associated with such a disease: two patients with apical gut epithelial cell autoantibodies showed no signs of intestinal lesion or diarrhea.
authors.affiliation: Department of Immunology, Hospital 12 de Octubre, Madrid, Spain.
authors.forename: J M
authors.initials: JM
authors.lastname: Martín-Villa
authors.forename: J R
authors.initials: JR
authors.lastname: Regueiro
authors.forename: D
authors.initials: D
authors.lastname: de Juan
authors.forename: P
authors.initials: P
authors.lastname: Pérez-Aciego
authors.forename: M
authors.initials: M
authors.lastname: Pérez Blas
authors.forename: J
authors.initials: J
authors.lastname: Manzanares
authors.forename: G
authors.initials: G
authors.lastname: Varela
authors.forename: A
authors.initials: A
authors.lastname: Arnaiz-Villena
mesh_terms: D000328:Adult; D001323:Autoantibodies / Q000032:analysis*; D001327:Autoimmune Diseases / Q000276:immunology; D002648:Child; D002675:Child, Preschool; D003168:Complement Fixation Tests; D004406:Dysgammaglobulinemia / Q000276:immunology; D004847:Epithelial Cells; D004848:Epithelium / Q000276:immunology; D005260:Female; D005455:Fluorescent Antibody Technique; D006801:Humans; D017098:IgA Deficiency; D017099:IgG Deficiency; D007410:Intestinal Diseases / Q000276:immunology; D007422:Intestines / Q000276:immunology*; D008297:Male; D013601:T-Lymphocytes / Q000276:immunology / Q000502:physiology*
chemical_list: D001323:Autoantibodies