Andersons pediatric cardiology 1389
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KawasakiDisease
AudreyDionne,AnnetteBaker,JaneW.Newburger
Abstract
Kawasakidiseasewasfirstdescribedin1967byTomisakoKawasaki,a
Japanesepediatrician.Hecharacterizedtheillness,thentermed
mucocutaneouslymphnodesyndrome,asincludinghighfever,
nonexudativeconjunctivitis,inflammationoftheoralmucosa,rash,cervical
adenopathy,andfindingsinthelimbs,includingswollenhandsandfeet,red
palmsandsoles,and,later,subungualpeeling.Initiallythoughttobeselflimited,thediseasewaslatershowntocausecoronaryarteryaneurysms
withriskofmyocardialinfarctionandsuddendeath.AlthoughKawasaki
diseasehasthehighestrelativeriskinJapanesechildren,ithasbeen
describedworldwideinchildrenofallracesandethnicities.Indeveloped
countries,Kawasakidiseasehassurpassedrheumaticfeverasthemost
frequentcauseofacquiredcardiacdiseaseinchildren.Inthischapter,we
summarizecurrentknowledgeabouttheetiologyandpathogenesis,
diagnosis,treatment,andnaturalhistoryofKawasakidisease.
Keywords
Kawasakidisease;coronaryarteryaneurysms;intravenous
immunoglobulin
Kawasakidiseasewasfirstdescribedin1967byTomisakoKawasaki,aJapanese
pediatrician.1Hecharacterizedtheillness,thentermedmucocutaneouslymph
nodesyndrome,asincludinghighfever,nonexudativeconjunctivitis,
inflammationoftheoralmucosa,rash,cervicaladenopathy,andfindingsinthe
limbs,includingswollenhandsandfeet,redpalmsandsoles,and,later,
subungualpeeling.Initiallythoughttobeself-limited,thediseasewaslater
showntocausecoronaryarteryaneurysmswithriskofmyocardialinfarctionand
suddendeath.AlthoughKawasakidiseasehasthehighestrelativeriskin
Japanesechildren,ithasbeendescribedworldwideinchildrenofallracesand
ethnicities.Indevelopedcountries2Kawasakidiseasehassurpassedrheumatic
feverasthemostfrequentcauseofacquiredcardiacdiseaseinchildren.This
chaptersummarizescurrentknowledgeabouttheetiologyandpathogenesis,
diagnosis,treatment,andnaturalhistoryofKawasakidisease.
Epidemiology
TheincidenceofKawasakidiseasediffersaccordingtoraceandethnicity.In
Japan,Kawasakidiseasehasanincidenceofapproximately265casesforevery
100,000childrenundertheageof4years.3TheincidenceofKawasakidisease
hascontinuedtoincreaseinJapan,eitherbecauseofagreaternumberofcasesor
increasedawarenessanddiagnosisofpatientswithincompletecriteria.Inthe
UnitedStates,theincidenceisapproximately21casesper100,000children,
higheramongAsiansandPacificIslanders(30per100,000),followedbynonHispanicAfrican-Americans(17per100,000),Hispanics(16per100,000),and
whites(12per100,000).4TheincidencerateintheUnitedStateshasbeenstable.
Kawasakidiseaseismostcommoninchildrenyoungerthan5years,butonequarterofcasesoccurinolderchildren.4Younginfantshavethehighestrateof
formationofcoronaryarteryaneurysmsandoftenpresentwithincomplete
features.Childrenolderthan8yearsalsohaveahigherrateofcoronaryartery
involvement.5–7Incontrasttoyounginfants,olderchildrengenerallypresent
withtypicalfindingsbutaremorelikelytohaveadelayeddiagnosis,inpart
becauseofthebeliefthatKawasakidiseaseoccursonlyinveryyoungchildren.
Malesareaffectedmorethanfemalesbyaratioof1.5to1,andtheillnessis
mostcommonduringthewinterandearlyspringmonths.4Datalinking
Kawasakidiseasetoantecedentexposuretocarpetcleaningortoviralinfections
oftherespiratorytracthavebeeninconsistent.7–13Otherstudieshavesuggested
thatKawasakidiseaseisassociatedwithresidencenearastandingbodyof
water,14antecedenteczema,15useofhumidifiers,13andperinatalexposure.16
InJapantherateofrecurrenceofthediseaseisapproximately3.5%,3with
halfofthecasesoccurringwithinayearoftheindexepisode.17Theoverallrisk
ofrecurrenceintheUnitedStateshasbeenestimatedat1.7%butishigher
(3.5%)inAsiansandPacificIslanders.18Patientswithcoronarycomplications
weremorelikelytohavearecurrence.17Siblingshavearelativeriskthatis10foldthatofthenormalJapanesepopulation,withhalfdevelopingthedisease
within10daysofthefirstcase.19Theriskofoccurrenceintwinsmaybeas
muchas100-foldhigherthanthatinthegeneralpopulation,witha14%riskof
concordance.19,20Ofnote,individualsofJapaneseancestryintheUnitedStates
arethoughttohavesimilarrisktothoseinJapan.
Coronaryarterydiseaseisresponsibleforalmostalldeathsinpatientswith
