Kawasaki Disease: A Maturational Defect in Immune Responsiveness

• Published in: The Journal of infectious diseases Vol. 180; no. 6; pp. 1869 - 1877
• Main Authors: Kuijpers, T. W., Wiegman, A., van Lier, R. A. W., Roos, M. T. L., Wertheim-van Dillen, P. M. E., Pinedo, S., Ottenkamp, J.
• Format: Journal Article
• Language: English
• Published: Chicago, IL The University of Chicago Press 01.12.1999; University of Chicago Press; Oxford University Press
• Subjects: Antibodies; Antibodies, Viral - blood; Biological and medical sciences; Blood; CD3 Complex - immunology; Child; Child, Preschool; Coronary Aneurysm; coronary artery; Female; Humans; Immune Tolerance; Immunoglobulin G - blood; Immunoglobulin G - metabolism; Infant; Infections; Lymphocyte Activation; Lymphocyte Subsets - immunology; Lymphocytes; Major Articles; Male; Matched-Pair Analysis; Measels mumps rubella vaccine; Measles; Measles Vaccine - immunology; Measles virus - immunology; Measles-Mumps-Rubella Vaccine; Medical sciences; Mucocutaneous lymph node syndrome; Mucocutaneous Lymph Node Syndrome - immunology; Mucocutaneous Lymph Node Syndrome - pathology; Mumps; Mumps Vaccine - immunology; Mumps virus - immunology; Prospective Studies; Rubella Vaccine - immunology; Rubella virus - immunology; Sarcoidosis. Granulomatous diseases of unproved etiology. Connective tissue diseases. Elastic tissue diseases. Vasculitis; T lymphocytes; T-Lymphocytes - immunology; Tetanus Toxoid - immunology; Vaccination; Vaccines, Combined - immunology; Vascular disease; Human; Kawasaki syndrome; Immunopathology; Vasculitis; Systemic disease; Cardiovascular disease; Autoimmune disease; Immune system
• ISSN: 0022-1899; 1537-6613
• DOI: 10.1086/315111

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.