川崎病进展课件

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KawasakiDisease:AnUpdateBevinWeeks,MDYaleUniversitySchoolofMedicine ObjectivesReviewthehistorical&clinicalfeaturesofa“common”butpoorlyunderstoodchildhooddiseaseExplorethedifferentialdiagnosisofthisillnessConsiderthemostimportantsequelaeofthisdiseaseDiscusstreatment&long-termmanagementrecommendations KawasakiDisease:AnUpdateHistoryofKawasakidiseaseEpidemiologyandetiologyPresentationanddiagnosisTreatmentChroniccardiovascularmanifestationsFollowupofpatientsQuestionsinthechronicmanagement KawasakiDisease:MucocutaneousLymphNodeSyndrome“Aself-limitedvasculitisofunknownetiologythatpredominantlyaffectschildrenyoungerthan5years.ItisnowthemostcommoncauseofacquiredheartdiseaseinchildrenintheUnitedStatesandJapan.”JaneBurns,MD**Burns,J.Adv.Pediatr.48:157.2001. HistoryofKawasakiDiseaseOriginalcaseobservedbyKawasakiJanuary19614y.o.boy,“diagnosisunknown”CAthrombosis1strecognized1965onautopsyofchildprev.dx’dw/MCOSFirstJapanesereportof50cases,1967FirstEnglishlanguagereportfromDr.Kawasaki1974,simultaneouslyrecognizedinHawaii WhatisKawasakiDisease?Idiopathicmultisystemdiseasecharacterizedbyvasculitisofsmall&mediumbloodvessels,includingcoronaryarteries EpidemiologyMedianageofaffectedchildren=2.3years80%ofcasesinchildren<4yrs,5%ofcasesinchildren>10yrsMales:females=1.5-1.7:1Recursin3%Positivefamilyhistoryin1%but13%riskofoccurrenceintwinsOverallU.S.in-hospitalmortality≈0.17% EpidemiologyAnnualincidenceof4-15/100,000childrenunder5yearsofageOver-representedinAsian-Americans,African-AmericansnextmostprevalentSeasonalvariationMorecasesinwinterandspringbutoccursthroughouttheyear TheBiggestQuestion:WhatistheEtiologyofKawasakiDisease? EtiologyInfectiousagentmostlikelyAge-restrictedsusceptiblepopulationSeasonalvariationWell-definedepidemicsAcuteself-limitedillnesssimilartoknowninfectionsNocausativeagentidentifiedBacterial,retroviral,superantigenicbacterialtoxinImmunologicresponsetriggeredbyoneofseveralmicrobialagents NewHavenCoronavirusIdentifiedanovelhumancoronavirusinrespiratorysecretionsfroma6-month-oldwithtypicalKawasakiDiseaseSubsequentlyisolatedfrom8/11(72.7%)ofKawasakipatients&1/22(4.5%)matchedcontrols(p=0.0015)Suggestsassociationbetweenviralinfection&KawasakidiseaseEsperF,et.JInfDis.2005;191:499-502 DiagnosticCriteriaFeverforatleast5daysAtleast4ofthefollowing5features:ChangesintheextremitiesEdema,erythema,desquamation2.Polymorphousexanthem,usuallytruncal3.Conjunctivalinjection4.Erythema&/orfissuringoflipsandoralcavity5.CervicallymphadenopathyIllnessnotexplainedbyotherknowndiseaseprocessModifiedfromCentersforDiseaseControl.KawasakiDisease.MMWR29:61-63,1980 AtypicalorIncompleteKawasakiDiseasePresentwith<4of5diagnosticcriteriaCompatiblelaboratoryfindingsStilldevelopcoronaryarteryaneurysmsNootherexplanationfortheillnessMorecommoninchildren<1yearofage2004AHAguidelinesoffernewevaluationandtreatmentalgorithm DifferentialDiagnosisInfectiousMeasles&GroupAbeta-hemolyticstrepcancloselyresembleKDBacterial:severestaphinfectionsw/toxinreleaseViral:adenovirus,enterovirus,EBV,roseola DifferentialDiagnosisInfectiousSpirocheteal:Lymedisease,LeptospirosisParasitic:ToxoplasmosisRickettsial:RockyMountainspottedfever,Typhus DifferentialDiagnosisImmunological/AllergicJRA(systemiconset)AtypicalARFHypersensitivityreactionsStevens-JohnsonsyndromeToxinsMercury PhasesofDiseaseAcute(1-2weeksfromonset)Febrile,irritable,toxicappearingOralchanges,rash,edema/erythemaoffeetSubacute(2-8weeksfromonset)Desquamation,mayhavepersistentarthritisorarthralgiasGradualimprovementevenwithouttreatmentConvalescent(Monthstoyearslater) Trager,J.D.NEnglJMed333(21):1391.1995. Trager,J.D.NEnglJMed333(21):1391.1995. Han,R.CMAJ162:807.2000. KawasakiDisease:S&SRespiratoryRhinorrhea,cough,pulmonaryinfiltrateGIDiarrhea,vomiting,abdominalpain,hydropsofthegallbladder,jaundiceNeurologicIrritability,asepticmeningitis,facialpalsy,hearinglossMusculoskeletalMyositis,arthralgia,arthritis KawasakiDisease:LabsEarlyLeukocytosisLeftshiftMildanemiaThrombocytopenia/ThrombocytosisElevatedESRElevatedCRPHypoalbuminemiaElevatedtransaminasesSterilepyuriaLateThrombocytosisElevatedCRP CardiovascularManifestationsofAcuteKawasakiDiseaseEKGchangesArrhythmiasAbnormalQwavesProlongedPRand/orQTintervalsLowvoltageST-T–wavechanges.CXR–cardiomegaly CardiovascularManifestationsofAcuteKawasakiDiseaseNoneSuggestiveofmyocarditis(50%)Tachycardia,murmur,galloprhythmsDisproportionatetodegreeoffever&anemiaSuggestiveofpericarditisPresentin25%althoughsymptomsarerareDistanthearttones,pericardialfrictionrub,tamponade CardiologyintheAcuteSettingUsuallyjusttodocumentbaselinecoronaryarterystatus–notanemergencyIfmyocarditissuspected–anemergencyCanhelpdiagnose“atypical”disease EchocardiographicFindingsMyocarditiswithdysfunctionPericarditiswithaneffusionValvarinsufficiencyCoronaryarterialchanges CoronaryArterialChanges15%to25%ofuntreatedpatientsdevelopcoronaryarterychanges3-7%iftreatedinfirst10daysoffeverwithIVIGMostcommonlyproximal,canbedistalLeftmain>LAD>Right CoronaryArterialChangesVaryinseverityfromechogenicityduetothickeningandedemaorasymptomaticcoronaryarteryectasiatogiantaneurysmsMayleadtomyocardialinfarction,suddendeath,orischemicheartdisease CoronaryAneurysmsSizeSmall=<5mmdiameterMedium=5-8mmGiant=≥8mmHighestriskforsequelaeShapeSaccularFusiform CoronaryAneurysms•PatientsmostlikelytodevelopaneurysmsYoungerthan6months,olderthan8yearsMalesFeverspersistforgreaterthan14daysPersistentlyelevatedESRThrombocytosisPtswhomanifests/sofcardiacinvolvement Snider,R.EchocardiographyinPediatricHeartDisease.1997. Bruckheimer,E.Circulation97:410.1998. Circulation103(2):335-336.2001. CoronaryAneurysmHistory50%regresstonormalbyecho25%becomesmaller25%donotregress7-20%developstenosisormyocardialinfarctionattributedtotheiraneurysms CoronaryAneurysmHistoryApproximately50%ofaneurysmsresolveSmallersizeFusiformmorphologyFemalegenderAgelessthan1yearGiantaneurysms(>8mm)worstprognosis CardiovascularSequelae0.3-2%mortalityrateduetocardiacdisease10%fromearlymyocarditisAneurysmsmaythrombose,causeMI/deathMIisprincipalcauseofdeathinKD32%mortalityMostofteninthefirstyearMajoritywhileatrest/sleepingAbout1/3asymptomatic AcuteKawasakiDisease:TreatmentIVIG:2g/kgasone-timedoseBeneficialeffect1streportedbyJapaneseMechanismofactionisunclearSignificantreductioninCAAinptstreatedwithIVIGplusaspirinvs.aspirinalone(15-25%3-5%)Efficacyunclearafterday10ofillness AcuteKawasakiDisease:TreatmentIVIG70-90%defervesce&showsymptomresolutionwithin2-3daysoftreatmentRetreatthosewithfailureofresponseto1stdoseorrecurrentsymptomsUpto2/3respondtoasecondcourse AcuteKawasakiDisease:TreatmentAspirinHighdose(80-100mg/kg/day)untilafebrilex48hrs&/ordecreaseinacutephasereactantsNeedhighdosesinacutephaseduetomalabsorptionofASADosageofASAinacutephasedoesnotseemtoaffectsubsequentincidenceofCAA AcuteKawasakiDisease:TreatmentAspirinDecreasetolowdose(3-5mg/kg/day)for6-8weeksoruntilplateletlevelsnormalizeNoevidencef/effectonCAAwhenusedaloneDuetopotentialriskofReyesyndromeinstructparentsaboutsymptomsofinfluenzaorvaricella AcuteKawasakiDisease:TreatmentAggressivesupportwithdiuretics&inotropesforsomepatientswithmyocarditisAntibioticswhileexcludingbacterialinfection AcuteKawasakiDisease:TreatmentConflictingdataaboutsteroidsReportsofhigherincidenceofaneurysms&moreischemicheartdzinptsw/aneurysmsCasereportofKDrefractorytoIVIGbutresponsivetohigh-dosesteroids&cyclosporineAOngoingNHLBImulticenterrandomizedplacebo-blindtrialinprogress PatientFollow-UpCategoriesFivecategoriesbasedoncoronaryarteriesfindingsNocoronarychangesatanystageofillnessTransientCAectasia,resolvedwithin6-8wksSmall/mediumsolitarycoronaryaneurysmOneormorelargeorgiantaneurysmsormultiplesmaller/complexaneurysmsinsameCA,withoutobstructionCoronaryarteryobstruction ManagementCategoriesPharmacologictherapyPhysicalactivityFollow-upanddiagnostictestingInvasivetesting I.NocoronarychangesatanystageofillnessPharmacologicTherapyNonebeyond6-8weeksPhysicalActivityNorestrictionsbeyond6-8weeksFollow-upanddiagnostictestingCVriskassessment,counseling@5yrintervalsInvasivetestingNonerecommended II.TransientCAectasia,resolvedwithin6-8wksPharmacologicTherapyNonebeyond6-8weeksPhysicalActivityNorestrictionsbeyond6-8weeksFollow-upanddiagnostictestingCVriskassessment,counseling@5yrintervalsInvasivetestingNonerecommended III.SingleSmallorMediumSizeAneurysmPharmacologicTherapyLowdoseASAuntilregressiondocumentedPhysicalActivityNonebeyond1st6-8weeksinpatients<11y.o.11-20y.o.:Restrictionsbasedonbiennialstresstest/myocardialperfusionscanContact/high-impactdiscouragediftakinganti-pltdrugsFollow-upanddiagnostictestingAnnualexam,echo,EKGCVriskassessment,counselingInvasivetestingAngiographyifsuggestionofischemia IV.AneurysmswithoutStenosisPharmacologicTherapyLong-termantiplatelettx&warfarinorLMWHPhysicalActivityRestrictionsbasedonstresstest/myocardialperfusionscanContact/high-impactavoidedduetoriskofbleedingFollow-upanddiagnostictestingBiannualexam,echo,EKGAnnualstresstest/myocardialperfusionscanInvasivetestingAngiography@6-12mos,sooner/repeatedifclinicallyindicatedElectiverepeatincertaincircumstances V.ObstructionPharmacologicTherapyLong-termlow-doseASA,±warfarinorLMWHifgiantaneurysmpersistsConsiderß-blockadetoreducemyocardialO2consumptionPhysicalActivityNocontactorhighimpactsportsOtheractivityguidedbystresstestingorperfusionscanFollow-upanddiagnostictestingBiannualexam,echoandEKGAnnualstresstest/myocardialperfusionscanInvasivetestingAngiographyindicatedtoassesslesionsandguidetherapy.Repeatangiographywithchangeinsymptoms.