KAWASAKI DISEASE

First described by Tomisaku

Kawasaki in 1967 in Japan.
Incidence in Japan : highest
Complication : coronary artery
aneurysm in 20 40%
Etiology : unknown
EPIDEMIOLOGY

Japan : highest : 125,000 cases till 1999

Japan and Korea : 50 -100 cases/year per
100,000 children < 5 yr
Male to female : 1.5 :1
Indonesia : > 100 cases, mostly Chinese
80 % below 4 yr, rare < 3 mo or > 8 yr
CLINICAL MANIFESTATIONS
ACUTE PHASE (First 10 days)
Fever (remittent), not responsive to antibiotic,
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)
Cardiovascular findings in 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 effusion,
myocard infarct
Thrombocytosis, peaking at 2 weeks />
Convalescent phase ( day 25 >)
Lasts till ESR and platelet count return to
normal. Deep transverse grooves (Beaus
lines) : 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)
Fever + 4 of remaining 5 criteria are
present : KD is probable.
Presence of coronary artery pathology
may be diagnostic even when < 4 criteria
are present
DIFFERENTIAL DIAGNOSIS
Measles
Stevens Johnson syndrome
Staphylococcal scalded skin syndrome
Drug reaction
Scarlet fever
Laboratory test not pathognomonic
Leukocytosis with a shift to the left
Mild to moderate anemia
CRP, ESR, alpha1 antitrypsin : increase
during acute phase
Thrombocytosis : subacute phase may
> 1,000,000 sometimes 2,000,000/mm3
Pyuria (due to urethritis)
Liver enzyme increase
Elevated CPK : myocard infarction
ECG
Low voltage QRS
ST elevation/depression
QTc >
Wide and deep Q wave : myocard infarct
Echocardiography
Most important
Detect coronary artery aneurysm and
cardiac dysfunction
May reveal coronary artery changes,
depressed LV function, regurgitation of
tricuspid, mitral, aortic valves and
pericardial effusion
N coronary size : baby 2 mm, toddler <3
mm, adolescent 5 mm
Catheterization
Selective
Large or multiple aneurysm
Sign of ischemia clinically or on ECG
Suspect stenosis
Management
No specific therapy available
Hospital admission with bed rest.
IVIG 2 g/kg BW in 10-12 hours : costly !!
Acetosal 80-100 mg/kgBW po 14 days or
2-3 days after fever subsides, 3-10
mg/kgBW once daily for 6-8 wks if echo N
 COURSE AND COMPLICATIONS
Self limiting except for the heart
IVIG : clinical improvement within 24 hrs and
reduce incidence of coronary aneurysm (CA)
Persisting CA ischaemic HD at young adult
Regressed CA intimal thickening & endothel
dysfunction premature atherosclerosis
Mortality 1-5 % decreasing
Lifelong monitoring needed ?