title: Autoimmune disease of exocrine organs in immunodeficient alymphoplasia mice: a spontaneous model for Sjögren's syndrome.
abstract: Mice homozygous for an autosomal recessive mutation aly (alymphoplasia) lack both lymph nodes and Peyer's patches, and show defects in both humoral and cellular immunity. Histopathological analysis revealed chronic inflammatory changes in exocrine organs such as the salivary gland, lacrimal gland, and pancreas of the homozygotes (aly/aly), but not the heterozygotes (aly/+). In these exocrine organs, mononuclear cells consisting mainly of CD4+ T cells infiltrate periductal areas, and, in some cases, the cell infiltration extended to lobules. The inflammatory changes in exocrine organs were transferred by a T cell-enriched fraction of spleen cells from homozygous animals. These results suggest that autoimmune mechanisms mediated by self-reactive T cells may be involved in the inflammatory lesions of various exocrine organs in the homozygous mice, although these mice show immunodeficiency. Inflammatory changes were also observed in the lung of the homozygotes. Since Sjögren's syndrome is characterized by diffuse lymphocyte infiltration in the periductal areas of the lacrimal and salivary glands and is occasionally associated with pulmonary disease, aly/aly mice may serve as a unique spontaneous model of Sjögren's syndrome.
authors.affiliation: Department of Medical Chemistry, Postgraduate School of Medicine, Kyoto University, Japan.
authors.forename: R
authors.initials: R
authors.lastname: Tsubata
authors.forename: T
authors.initials: T
authors.lastname: Tsubata
authors.forename: H
authors.initials: H
authors.lastname: Hiai
authors.forename: R
authors.initials: R
authors.lastname: Shinkura
authors.forename: R
authors.initials: R
authors.lastname: Matsumura
authors.forename: T
authors.initials: T
authors.lastname: Sumida
authors.forename: S
authors.initials: S
authors.lastname: Miyawaki
authors.forename: H
authors.initials: H
authors.lastname: Ishida
authors.forename: S
authors.initials: S
authors.lastname: Kumagai
authors.forename: K
authors.initials: K
authors.lastname: Nakao
authors.forename: T
authors.initials: T
authors.lastname: Honjo
mesh_terms: D019264:Adoptive Transfer; D000818:Animals; D001327:Autoimmune Diseases / Q000235:genetics*; D002465:Cell Movement / Q000276:immunology; D004268:DNA-Binding Proteins; D004195:Disease Models, Animal; D005088:Exocrine Glands / Q000276:immunology*; D005260:Female; D006720:Homozygote; D007153:Immunologic Deficiency Syndromes / Q000235:genetics* / Q000473:pathology; D008297:Male; D051379:Mice; D008817:Mice, Mutant Strains; D011506:Proteins / Q000032:analysis; D012859:Sjogren's Syndrome / Q000235:genetics* / Q000473:pathology
chemical_list: D004268:DNA-Binding Proteins; D011506:Proteins; C499308:Rag2 protein, mouse; C064655:V(D)J recombination activating protein 2