title: Kawasaki disease: a maturational defect in immune responsiveness.
abstract: Kawasaki disease (KD), an acute febrile disease in children of unknown etiology, is characterized by a vasculitis that may result in coronary artery aneurysms (CAAs). In new patients with KD, a selective and prolonged T cell unresponsiveness to activation via the T cell antigen receptor CD3 was observed, whereas proliferation to other stimuli was intact. This "split T cell anergy" delineated KD from other pediatric infections and autoimmune diseases and correlated with CAA formation (P<.001). A transient immune dysfunction was also suggested by an incomplete responsiveness to measles-mumps-rubella (MMR) vaccination in patients with KD versus controls (P<.0001; odds ratio, 15.6; 95% confidence interval, 4.8-51.1), which was overcome by revaccination(s). The reduced responsiveness to MMR in patients with KD suggests a subtle and predetermining immune dysfunction. An inherent immaturity to clear certain antigens may be an important cause that precipitates KD and the immune dysregulation during acute disease.
authors.affiliation: Emma Children's Hospital, Academic Medical Center, 1105 AZ Amsterdam, The Netherlands. T.W.Kuijpers@amc.uva.nl
authors.forename: T W
authors.initials: TW
authors.lastname: Kuijpers
authors.forename: A
authors.initials: A
authors.lastname: Wiegman
authors.forename: R A
authors.initials: RA
authors.lastname: van Lier
authors.forename: M T
authors.initials: MT
authors.lastname: Roos
authors.forename: P M
authors.initials: PM
authors.lastname: Wertheim-van Dillen
authors.forename: S
authors.initials: S
authors.lastname: Pinedo
authors.forename: J
authors.initials: J
authors.lastname: Ottenkamp
mesh_terms: D000914:Antibodies, Viral / Q000097:blood; D017252:CD3 Complex / Q000276:immunology; D002648:Child; D002675:Child, Preschool; D003323:Coronary Aneurysm; D005260:Female; D006801:Humans; D007108:Immune Tolerance; D007074:Immunoglobulin G / Q000097:blood / Q000378:metabolism; D007223:Infant; D008213:Lymphocyte Activation; D016131:Lymphocyte Subsets / Q000276:immunology; D008297:Male; D016555:Matched-Pair Analysis; D008458:Measles Vaccine / Q000276:immunology; D008459:Measles virus / Q000276:immunology; D022542:Measles-Mumps-Rubella Vaccine; D009080:Mucocutaneous Lymph Node Syndrome / Q000276:immunology* / Q000473:pathology; D009108:Mumps Vaccine / Q000276:immunology; D009109:Mumps virus / Q000276:immunology; D011446:Prospective Studies; D012411:Rubella Vaccine / Q000276:immunology; D012412:Rubella virus / Q000276:immunology; D013601:T-Lymphocytes / Q000276:immunology*; D013745:Tetanus Toxoid / Q000276:immunology; D017778:Vaccines, Combined / Q000276:immunology
chemical_list: D000914:Antibodies, Viral; D017252:CD3 Complex; D007074:Immunoglobulin G; D008458:Measles Vaccine; D022542:Measles-Mumps-Rubella Vaccine; D009108:Mumps Vaccine; D012411:Rubella Vaccine; D013745:Tetanus Toxoid; D017778:Vaccines, Combined