title: Distribution of circulating cells in systemic juvenile idiopathic arthritis across disease activity states.
abstract: Juvenile idiopathic arthritis (JIA) encompasses a group of chronic childhood arthritides of unknown etiology. One subtype, systemic JIA (SJIA), is characterized by a combination of arthritis and systemic inflammation. Its systemic nature suggests that clues to SJIA pathogenesis may be found in examination of peripheral blood cells. To determine the immunophenotypic profiles of circulating mononuclear cells in SJIA patients with different degrees of disease activity, we studied PBMC from 31 SJIA patients, 20 polyarticular JIA patients (similar to adult rheumatoid arthritis), and 31 age-matched controls. During SJIA disease flare, blood monocyte numbers were increased, whereas levels of myeloid dendritic cells (DC) and gammadelta T cells were reduced. At both flare and quiescence, increased levels of CD14 and CD16 were found on SJIA monocytes. Levels of CD16-DC were elevated at SJIA quiescence compared both to healthy controls and to SJIA subjects with active disease. Overall, our findings suggest dysregulation of innate immunity in SJIA and raise the possibility that quiescence represents a state of compensated inflammation.
authors.affiliation: Program in Immunology, Division of Human Gene Therapy, Department of Pediatrics, Stanford University School of Medicine, 300 Pasteur Drive, Stanford, CA 94305-5164, USA.
authors.forename: Claudia
authors.initials: C
authors.lastname: Macaubas
authors.forename: Khoa
authors.initials: K
authors.lastname: Nguyen
authors.forename: Chetan
authors.initials: C
authors.lastname: Deshpande
authors.forename: Carolyn
authors.initials: C
authors.lastname: Phillips
authors.forename: Ariana
authors.initials: A
authors.lastname: Peck
authors.forename: Tzielan
authors.initials: T
authors.lastname: Lee
authors.forename: Jane L
authors.initials: JL
authors.lastname: Park
authors.forename: Christy
authors.initials: C
authors.lastname: Sandborg
authors.forename: Elizabeth D
authors.initials: ED
authors.lastname: Mellins
mesh_terms: D000293:Adolescent; D001171:Arthritis, Juvenile / Q000097:blood* / Q000276:immunology*; D001402:B-Lymphocytes / Q000276:immunology; D002469:Cell Separation; D002648:Child; D002675:Child, Preschool; D003713:Dendritic Cells / Q000276:immunology*; D005260:Female; D005434:Flow Cytometry; D006801:Humans; D016130:Immunophenotyping; D007694:Killer Cells, Natural / Q000276:immunology; D007963:Leukocytes, Mononuclear / Q000276:immunology*; D008297:Male; D009000:Monocytes / Q000276:immunology*; D016176:T-Lymphocyte Subsets / Q000276:immunology; D013601:T-Lymphocytes / Q000276:immunology