Kawasaki disease: a maturational defect in immune responsiveness

T.W. Kuijpers; A. Wiegman; R.A.W. van Lier; M.T.L. Roos; P.M.E. Wertheim-van Dillen; S. Pinedo; J. Ottenkamp

doi:https://doi.org/10.1086/315111

Kawasaki disease: a maturational defect in immune responsiveness

T.W. Kuijpers, A. Wiegman, R.A.W. van Lier, M.T.L. Roos, P.M.E. Wertheim-van Dillen, S. Pinedo, J. Ottenkamp

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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

Original language	Undefined/Unknown
Pages (from-to)	1869-1877
Journal	The Journal of Infectious Diseases
Volume	180
Issue number	6
DOIs	https://doi.org/10.1086/315111
Publication status	Published - 1999

Keywords

AMC wi-co

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https://doi.org/10.1086/315111

https://pure.uva.nl/ws/files/3111947/6162_74579y.pdf

Cite this

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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",

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T1 - Kawasaki disease: a maturational defect in immune responsiveness

AU - Kuijpers, T.W.

AU - Wiegman, A.

AU - van Lier, R.A.W.

AU - Roos, M.T.L.

AU - Wertheim-van Dillen, P.M.E.

AU - Pinedo, S.

AU - Ottenkamp, J.

PY - 1999

Y1 - 1999

N2 - 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

AB - 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

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