Kawasaki disease: what should we know?

Dr dr Najib Advani SpA(K) MMed(Paed)

IKA FKUI-RSCM

najib.advani@gmail.com
0813 1515 9500
• First described by Tomisaku Kawasaki in
1967 in Japan.
• Complication : coronary artery aneurysms in
20 – 40% of patients
• Etiology : unknown
• Systemic vasculitis
Kawasaki Disease
 EPIDEMIOLOGY

The commonest acquired heart disease in

children in developed countries
 Japan: highest, 200 per 100,000 children < 5 yr
 Male to female: 1.6 : 1 (Advani 2014)
 Indonesia
Estimated incidence 5000 per year, diagnosed
100/yr (Advani 2006, Advani et al 2008)
 71 % below 5 year, youngest 33 days, oldest 16 year
(Advani 2014)
Distribusi pasien PK menurut usia
n=667 (2003-2013)
 Total pasien per bulan 2003-2012 n=598
70

60

50

40

30

20

10

0
Jan Feb Mar Apr Mei Juni Juli Agustus Sept Okt Nov Des
Distribusi pasien baru PK pertahun
(n=598)
Kawasaki disease in Indonesia : an early report
Najib Advani, Rubiana Sukardi, Sudigdo Satroasmoro, Bondan Lukito, Andreas Liando

There has not been any report on Kawasaki disease case series from
Indonesia. In order to see the pattern of KD patients, we did a
retrospective study at two hospitals Jakarta, Indonesia. We found 27
patients with clinically confirmed diagnosis of Kawasaki disease
…………………………….

Presented at the Eighth International Symposium on Kawasaki Disease

San Diego, USA, February 2005

 CLINICAL MANIFESTATIONS
ACUTE PHASE (First 10 days)
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
Fever (remittent), not responsive to antibiotics, may
persist for 1-2 / 3-4 wks
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
 My HEART
Clinical manifestations of KD
M ucosal changes : erythema
H and and foot changes: erythema,
edema
E ye changes : conjunctivitis
A denopathy : unilateral
R ash : polymorph exanthem
T emperature : remittent
Cardiovascular findings during acute phase

Tachycardia
Murmur / gallop
Cardiomegaly
Pericardial effusion
LV dysfunction
ECG changes : PR interval >, low QRS voltage
ST depression/elevation
Subacute phase (day 11-25)
Desquamation: tips of fingers and toes
Rash, fever, lymphadenopathy disappear
Significant cardiovascular changes : coronary
aneurysm, pericardial eff, myocard infarct
Thrombocytosis, peaking at 2 weeks />
Convalescent phase (day > 25)
Lasts till ESR and platelet count return to
normal. Deep transverse grooves (Beau’s
line) : finger nails and toenails
DIAGNOSTIC CRITERIA FOR KD

1. Remittent fever for 5 days/more

2. Bilateral conjunctival injection (no exudate)
3. Changes in the mouth and lips : strawberry tongue,
diffuse reddening of oral cavity, erythema and cracking of
lips.
4. Changes in the hands and feet : erythema and edema
5. Polymorphous exanthem
6. Unilateral cervical lymphadenopathy (>1.5 cm)
 Diagnostic criteria
• Fever plus 4 of the 5 other criteria allows for
diagnosis
• Fever plus fewer than 4 of 5 other criteria can
be diagnosed as KD if coronary artery disease
is detected (incomplete KD)
• Other possible diagnoses should be excluded
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
KD should be considered in DD/ of every child
with fever of at least several days’ duration,
rash, and nonpurulent conjunctivitis
Laboratory test : not pathognomonic
• Leukocytosis with a shift to the left
• Mild to moderate anemia
• CRP, ESR 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 : myocard infarct
ST depression and sinus tachycardia at acute stage of KD before IVIG
Three days post IVIG, normal ECG
Echocardiography
• Mandatory
• Detect coronary artery aneurysm and cardiac
dysfunction
• May reveal coronary artery changes, depressed
LV function, regurgitation tricuspid, mitral,
aortic and pericardial effusion
• N coronary size : Z score
Catheterization
• Selective
• Large or multiple aneurysm
• Sign of ischemia clinically or in ECG
• Suggest stenosis
 Invasive treatment

• Coronary Artery Bypass Graft (CABG) for

obstructive lesion
• Baloon angioplasty : not successful
• Stent placement : in older children
• Cardiac transplantation : last choice
 DIFFERENTIAL DIAGNOSIS

• Measles
• Stevens Johnson syndrome
• Staphylococcal scalded skin syndrome
• Drug reaction
• Scarlet fever
• Roseola infantum
Diagnosis should avoid :
• Over diagnosis
Actually not Kawasaki but diagnosed as Kawasaki
• Under diagnosis
Actually Kawasaki but undiagnosed

Need to have a good knowledge on DD/ of KD

 Management
All KD patients should be hospitalized, consult pediatric cardiologist if possible

IGIV 2 g/kgBW single dose within 10-12 hours

Aspirin 80-100 mg/kgBW/day, orally divided into 4 doses till 2-3 days after fever
subsides, then
 3-5 mg/kgBW/day single dose untill no aneurysms detected by echocardiography ,
at least for 6 weeks

Fever persists >36-48 hours after completion of IVIG -> repeat IVIG if necessary
(reevaluate diagnosis, no other source of fever)

Newburger JW, dkk. Pediatrics. 2004;114:1708-33.

Dummer KB, dkk. Pediatr Cardiol. 2004;19:129-35.
 Outcome

Without coronary aneurysms

Total recovery

Myocarditis, pericardial effusion

Improve within 1 month

With coronary aneurysms

Outcome depends on diameter of aneurysms

Advani, thesis, 2014

Belay ED, Pediatr Infect Dis J. 2006;25:245-249.
Kato H, dkk. Circulation. 1996;94:1379-85..
 Outcome

Small aneurysm (< 5 mm)

Mostly regress within 2 years

Moderate aneurysms (5-8 mm)

Mostly regress within 5 years

Giant aneurysms (>8mm)

Unlikely to regress  thrombosis or stenosis may follow

years later

Regressed aneurysms  intimal thickening and endothelial dysfunction 

atherosclerotic lesion  long-term follow up needed?

Advani, thesis, 2014

Belay ED, Pediatr Infect Dis J. 2006;25:245-249.
Kato H, dkk. Circulation. 1996;94:1379-85..
 Conclusion
KD is a vasculitis of unknown etiology
Diagnosis of KD is based on clinical findings, lab tests are
not specific but may support the diagnosis
KD should be considered in DD/ of every child with fever
of at least several days’ duration, rash, and nonpurulent
conjunctivitis
All patients with KD must be hospitalized and consulted
to a pediatric cardiologist who is familiar with KD
IVIG 2 g/kg BW for 10-12 hours is the treatment of
choice, best given on day 5-10
Coronary aneurysm occurs in 15-25% of untreated cases
Outcome depends on the severity of coronary aneurysms