Kawasaki Disease

Noormanto
Division of Pediatric Cardiology,
Departement of Pediatric, Faculty of Medicine
GMU / Sardjito General Hospital Yogyakarta
First described by Tomisaku Kawasaki in
1967 in Japan.
Complication : coronary artery aneurysm
in 20 – 40%
Etiology : unknown
Systemic vasculitis
 Epidemiology

The commonest acquired heart disease in

children in developed countries
Japan : highest 125,000 case till 1999
Asia esp Japan and Korea : 100/ year
per 100,000 children below 5 yr
Male to female : 1.5 : 1
Indonesia : > 100 cases, mostly Chinese
Incidence 5000 per year
80 % below 4 yr, rare < 3 mo or > 8 yr
 Clinical Manifestations
ACUTE PHASE (First 10 days)
Fever (remittent), not responsive to antibiotics,
may persist for 1-2 / 3-4 wks
Conjunctivitis, bilateral, non exudative
Changes in mouth and lips : strawberry tongue,
red oral cavity, erythema and cracked lips
Changes in the hands and feet : erythema and
edema
Polymorphous exanthem
Cervical lymphadenopathy, unilateral (>1.5 cm)
Other associated findings (acute phase)
Sterile pyuria (60 %)
Liver dysfunction (40%)
Arthritis of large joints (30%)
Aseptic meningitis (25%)
Abdominal pain with diarrhea
Hydrops of gallbladder with jaundice
CNS symptoms (irritable, lethargic, semicoma)
BCG scar : redness and crust
Cardiovascular findings during acute phase

Tachycardia
Murmur / gallop
Cardiomegaly
Pericardial effusion
LV dysfunction
ECG changes : PR interval >, low QRS voltage
ST depression/elevation
 Laboratory test
not pathognomonic
Leukocytosis with a shift to the left
Mild to moderate anemia
CRP, ESR, alpha1 antitrypsin : during acute
phase
Thrombocytosis : subacute phase may
> 1,000,000 sometimes 2,000,000/mm3
Pyuria (due to urethritis)
Liver enzyme increase, hypoalbuminemia
Elevated CPK : myocard infarction
ECG
Low voltage QRS
ST elevation/depression
QTc >
Wide and deep Q wave
Echocardiography
Mandatory
Detect coronary artery aneurysm and cardiac
dysfunction
May reveal coronary artery changes, depressed
LV function, regurgitation tricuspid, mitral, aortic
and pericardial effusion
Normal coronary size : baby 2 mm, toddler <3
mm, adolescent 5 mm
DIAGNOSTIC CRITERIA

Remittent fever for 5 days/more

Bilateral conjunctival injection (no exudate)
Changes in the mouth and lips : strawberry
tongue, diffuse reddening of oral cavity,
erythema and cracking of lips.
Changes in the hands and feet : erythema and
edema
Polymorphous exanthem
Unilateral cervical lymphadenopathy (>1.5 cm)
Fever + ≥4 of remaining five criteria are
present: Kawasaki disease is probable.
Presence of coronary artery pathology may be
diagnostic even when < 4 criteria are present:
incomplete Kawasaki disease, mostly in babies.
Incomplete Kawasaki disease should be
suspected in all children with: unexplained fever
≥ 5 days + 2-3 diagnostic criteria
Not all of the clinical features may present at a
single point in time  watchful waiting is
sometimes necessary before a diagnosis can be
made
DIFFERENTIAL DIAGNOSIS
Measles
Stevens Johnson syndrome
Staphylococcal scalded skin syndrome
Drug reaction
Scarlet fever
Roseola infantum
 Treatment
Hospital admission with bed rest
Consult experienced pediatric cardiologist
IVIG 2 g/kg BW in 10-12 hours : costly !!
10 % fail  repeat with same dose
2 times IVIG fail  methylprednisolone,
30 mg/kg BW for 2-3 hours once daily for
1-3 days
Acetosal 80-100 mg/kg BW po 14 days or 2-3
days after fever subsides,  3-10 mg/kgBW
once daily for 6-8 wks if echo normal
IVIG administration
• Relatively save
• Given during acute phase (less than day 10 of
onset)
• 90 % responsive to single dose
• After day 10 : given only if inflammatory signs
persist
• Monitor HR and BP in 30 minutes, 1 hour, and
later every 2 hours.
• Increase speed of administration gradually
 Course and complications

Self limiting
IVIG : clinical improvement within 24 hours and reduce
incidence of Coronary Aneurysm (C.A)
Arterial remodeling or revascularization may occur
coronary artery  infarct
Mortality 1-5 %  decreasing
Persisting coronary artery  ischaemic heart disease at
young adult age
Regressed coronary artery  intimal thickening &
endothelium dysfunction  premature atherosclerosis
Lifelong monitoring needed?
Risk Factors for coronary aneurysm

Male
Age < 1 year
Other signs or symptoms of pericardial,
myocardial or endocardial involvement,
including arrhythmias
Prolonged period of inflammation,
including fever > 10 days
Recurrence of fever after an afebrile
period of at least 24 hours