Kawasaki disease: a maturational defect in immune responsiveness.
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.
Kuijpers
T W
TW
Emma Children's Hospital, Academic Medical Center, 1105 AZ Amsterdam, The Netherlands. T.W.Kuijpers@amc.uva.nl
Wiegman
A
A
van Lier
R A
RA
Roos
M T
MT
Wertheim-van Dillen
P M
PM
Pinedo
S
S
Ottenkamp
J
J
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
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