Kawasaki Kawasaki DiseaseDiseaseVaishali Soneji Lafita, MDVaishali Soneji Lafita, MD

Presentation – Patient Presentation – Patient 11 10 years old male with Kawasaki 10 years old male with Kawasaki

DiseaseDisease Possible mildly ectatic posterior Possible mildly ectatic posterior

descending coronary artery on Echo.descending coronary artery on Echo. CTA was performed.CTA was performed.

Heart rate controlHeart rate controlContrast enhanced axial images of the Contrast enhanced axial images of the

coronary arteriescoronary arteriesMultiplanar and 3D reformatsMultiplanar and 3D reformats

CTA – Patient 1CTA – Patient 1

CTA – Patient 1CTA – Patient 1

CTA – Patient 1CTA – Patient 1

CTA – Patient 1CTA – Patient 1

Presentation – Patient Presentation – Patient 22 13 years old male with Kawasaki 13 years old male with Kawasaki

Disease and known coronary Disease and known coronary aneurysm presents for CTA.aneurysm presents for CTA.Heart rate controlHeart rate controlContrast enhanced axial images of Contrast enhanced axial images of

the coronary arteriesthe coronary arteriesMultiplanar and 3D reformatsMultiplanar and 3D reformats

CTA – Patient 2CTA – Patient 2

CTA – Patient 2CTA – Patient 2

CTA – Patient 2CTA – Patient 2

Presentation – Patient Presentation – Patient 33 13 years old female with 13 years old female with

Kawasaki Disease and known Kawasaki Disease and known coronary aneurysm diagnosed on coronary aneurysm diagnosed on Echocardiogram presents for CTA.Echocardiogram presents for CTA.

CTA – Patient 3CTA – Patient 3

Kawasaki SyndromeKawasaki Syndrome

AKA – Mucocutaneous Lymph AKA – Mucocutaneous Lymph Node SyndromeNode Syndrome

First characterized in 1967 by Dr. First characterized in 1967 by Dr. Tomisaku Kawasaki of TokyoTomisaku Kawasaki of Tokyo

Multisystem vasculitisMultisystem vasculitis Self limited Self limited Leading cause of acquired heart Leading cause of acquired heart

disease in the US.disease in the US.

EpidemiologyEpidemiology

Syndrome of young childrenSyndrome of young children– >80% are < 4y/o>80% are < 4y/o– Most are between 1-2y/oMost are between 1-2y/o– Very rare > 8y/oVery rare > 8y/o

Males > femalesMales > females Japanese and Korean ancestry > Japanese and Korean ancestry >

other ethnicitiesother ethnicities 4,000-8,000 cases/yr4,000-8,000 cases/yr

EtiologyEtiology

UnknownUnknown

Leading hypothesisLeading hypothesis

– Infectious with immune-mediated Infectious with immune-mediated

reactionreaction

– No single etiologic agent identifiedNo single etiologic agent identified

Clinical ManifestationClinical Manifestationand Diagnosisand Diagnosis No recognized prodromeNo recognized prodrome Acute onsetAcute onset

– Begins with feverBegins with fever– Other symptoms begin within 2-5 daysOther symptoms begin within 2-5 days

SyndromeSyndrome– Fever for 5d + 4/5 symptomsFever for 5d + 4/5 symptoms– Fever for 5d + 3/5 symptoms + Fever for 5d + 3/5 symptoms +

coronary artery abnormalitiescoronary artery abnormalities

Signs/symptomsSigns/symptoms

1.1. Nonpurulent bilateral conjunctivitsNonpurulent bilateral conjunctivits2.2. Oropharyngeal changesOropharyngeal changes

– Erythema, redness, cracking, peeling, injected Erythema, redness, cracking, peeling, injected pharynx, strawberry tonguepharynx, strawberry tongue

3.3. Polymorphous erythematous rashPolymorphous erythematous rash– Most evident with feverMost evident with fever– Primarily on trunk, may be pruriticPrimarily on trunk, may be pruritic

4.4. Cervical lymphadenopathyCervical lymphadenopathy– Often unilateralOften unilateral– One node >1.5cmOne node >1.5cm

5.5. Feet/Hand changesFeet/Hand changes– Erythema (palms/soles), edema, induration, Erythema (palms/soles), edema, induration,

desquamationdesquamation

Signs/SymptomsSigns/Symptoms

Cardiac findingsCardiac findings– Leading cause of morbidity and Leading cause of morbidity and

mortalitymortality– May involve pericardium, May involve pericardium,

myocardium, endocardium, valves, myocardium, endocardium, valves, coronary arteriescoronary arteries

Signs/SymptomsSigns/Symptoms

20-25%20-25% of untreated of untreated pts develop coronary pts develop coronary artery artery abnormalities/aneurysabnormalities/aneurysmsms– Within 4 weeks of onsetWithin 4 weeks of onset– Dilation can be Dilation can be

detected at 10ddetected at 10d– Peak 18-25dPeak 18-25d

Giant aneurysm Giant aneurysm >8mm>8mm– Greatest risk Greatest risk

thrombosis, stenosis, MIthrombosis, stenosis, MI

ComplicationsComplications

Cardiac manifestations can be Cardiac manifestations can be deadlydeadly– Most deaths occur between 2-12 Most deaths occur between 2-12

weeks post illness onsetweeks post illness onset Coronary aneurysm thrombosis – MICoronary aneurysm thrombosis – MI Aneurysm ruptureAneurysm rupture Myocarditis - CHFMyocarditis - CHF

PredictingPredictingCoronary Artery Coronary Artery abnormalitiesabnormalities

Harada scoreHarada score1.1. White blood cell count > 12KWhite blood cell count > 12K2.2. Platlet count < 350KPlatlet count < 350K3.3. CRP >3+CRP >3+4.4. Hct < 35%Hct < 35%5.5. Albumin < 3.5 g/dLAlbumin < 3.5 g/dL6.6. Age < 12 monthsAge < 12 months7.7. Male sexMale sex

Used to determine managementUsed to determine management

TreatmentTreatment

Decrease inflammationDecrease inflammation Prevent thrombosisPrevent thrombosis Overall goalOverall goal

– Protect the myocardium and coronary Protect the myocardium and coronary arteriesarteries

Modes of treatmentModes of treatment– IVIGIVIG– SteroidsSteroids– TNF –alpha antagonistsTNF –alpha antagonists– AspirinAspirin– abciximababciximab

Cardiac findings : Cardiac findings : ImagingImaging EchocardiographyEchocardiography

– Unclear sensitivity and specificityUnclear sensitivity and specificity Coronary AngiographyCoronary Angiography Intravascular Ultrasound (IVUS)Intravascular Ultrasound (IVUS) Transesophageal echocardiographyTransesophageal echocardiography MRAMRA CTACTA

Cardiac Cardiac findings :Imagingfindings :Imaging

EchocardiogramEchocardiogram 11stst at time of diagnosis at time of diagnosis Number, location and classification of Number, location and classification of

aneurysmsaneurysms– Small, medium or giantSmall, medium or giant

Repeat echo 2-3 and 6-8 weeks after Repeat echo 2-3 and 6-8 weeks after onset of the diseaseonset of the disease– If both normal – no further echos neededIf both normal – no further echos needed

If abnormalIf abnormal– Severity determines frequency and type of Severity determines frequency and type of

follow-upfollow-up

Cardiac Cardiac findings :Imagingfindings :Imaging

Management of Management of AneurysmsAneurysms Small to medium:Small to medium: long term Aspirin, long term Aspirin,

no activity restriction, stress test in no activity restriction, stress test in older children if suggests stenosis→ older children if suggests stenosis→ coronary angiographycoronary angiography

Multiple, small to medium or Multiple, small to medium or giant: giant: Aspirin with/without warfarin, Aspirin with/without warfarin, >10y stress test with myocardial >10y stress test with myocardial perfusion scan if coronary obstruction→ perfusion scan if coronary obstruction→ bypass grafting, angioplastybypass grafting, angioplasty

SummarySummary

Self limited systemic vasculitisSelf limited systemic vasculitis Leading cause of acquired heart Leading cause of acquired heart

disease in the US.disease in the US. Morbidity and mortality from Morbidity and mortality from

cardiac involvement.cardiac involvement. Imaging plays critical role in Imaging plays critical role in

diagnosis and management of diagnosis and management of cardiac involvement. cardiac involvement.

ReferencesReferences

J. W. Newburger, M. Takahashi, M. A. Gerber, M. H. Gewitz, L. Y. Tani, J. C. Burns, S. T. Shulman, A. F. Bolger, P. Ferrieri, R. S. Baltimore, W. R. Wilson, L. M. Baddour, M. E. Levison, T. J. Pallasch, D. A. Falace, and K. A. TaubertDiagnosis, Treatment, and Long-Term Management of Kawasaki Disease: A Statement for Health Professionals From the Committee on Rheumatic Fever, Endocarditis, and Kawasaki Disease, Council on Cardiovascular Disease in the Young, American Heart AssociationPediatrics, December 1, 2004; 114(6): 1708 - 1733.

http://www.emedicine.com/EMERG/topic811.htmhttp://www.emedicine.com/EMERG/topic811.htm http://www.cdc.gov/ncidod/diseases/kawasaki/index.hthttp://www.cdc.gov/ncidod/diseases/kawasaki/index.ht

mm http://www.http://www.americanheartamericanheart.org/presenter..org/presenter.jhtmljhtml

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