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2016 ; 186
(2
): 134-143
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Kawasaki disease: a matter of innate immunity
#MMPMID27342882
Hara T
; Nakashima Y
; Sakai Y
; Nishio H
; Motomura Y
; Yamasaki S
Clin Exp Immunol
2016[Nov]; 186
(2
): 134-143
PMID27342882
show ga
Kawasaki disease (KD) is an acute systemic vasculitis of childhood that does not
have a known cause or aetiology. The epidemiological features (existence of
epidemics, community outbreaks and seasonality), unique age distribution and
clinical symptoms and signs of KD suggest that the disease is caused by one or
more infectious environmental triggers. However, KD is not transmitted
person-to-person and does not occur in clusters within households, schools or
nurseries. KD is a self-limited illness that is not associated with the
production of autoantibodies or the deposition of immune complexes, and it rarely
recurs. Regarding the underlying pathophysiology of KD, innate immune activity
(the inflammasome) is believed to play a role in the development of KD
vasculitis, based on the results of studies with animal models and the clinical
and laboratory findings of KD patients. Animal studies have demonstrated that
innate immune pathogen-associated molecular patterns (PAMPs) can cause vasculitis
independently of acquired immunity and have provided valuable insights regarding
the underlying mechanisms of this phenomenon. To validate this concept, we
recently searched for KD-specific PAMPs and identified such molecules with high
specificity and sensitivity. These molecules have structures similar to those of
microbe-associated molecular patterns (MAMPs), as shown by liquid
chromatography-tandem mass spectrometry. We propose herein that KD is an innate
immune disorder resulting from the exposure of a genetically predisposed
individual to microbe-derived innate immune stimulants and that it is not a
typical infectious disease.