Kawasaki Disease
Mucocutaneous lymph node syndrome
Ma Lian
Introduction


Kawasaki disease
(KD) is a common
vasculitic disorder
usually seen in
children below 5
years of age
The leading cause
of acquired heart
disease in
children
Kawaski T.
Acute febrile mucocutaneous syndrome
with lymphoid involvement with specific
desquamation of the fingers and toes in
children: clinical observations of 50 cases.
Jpn J Allergol.1967; 16 :178 –222
China kawasaki disease
Epidemiologic study of admitted
children with Kawasaki disease in
Beijing from 1995 to 1999 :The
incidence of KD in Beijing is lower
than that reported in Japan, similar
to the incidence in the United States
and higher than in those other
Western countries.
Etiology

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No one knows what causes Kawasaki
disease.
But it is thought to start from an
infection or from exposure to some
toxin.
There is no firm evidence that the
disease can spread from one person
to another.
Superantigen-producing bacteria.
Histroy
Kawasaki disease has 3 stages,
as follows
 Acute stage (1-11 d)
 Subacute stage (11-30 d)
 Convalescent/chronic phase
(>30 d)
Acute stage (1-11 d)
1. High fever (temperature >39℃)
2. Nonexudative bilateral conjunctivitis
(90%)
3. Polymorphous erythematous rash
4. Acral erythema and edema that
impede ambulation
5. Strawberry tongue and lip fissures
6. Lymphadenopathy (75%), generally a
single, enlarged, nonsuppurative cervical
node measuring approximately 1.5 cm
Acute stage
Nonexudative bilateral
conjunctivitis (90%)
Acute stage

Polymorphous erythematous
rash
Acute stage

Acral erythema and edema that
impede ambulation
Acute stage

Strawberry tongue and lip
fissures
Acute stage

Lymphadenopathy (75%),
generally a single, enlarged,
nonsuppurative cervical node
measuring approximately 1.5 cm
Acute stage (1-11 d)
7. Hepatic, renal, and gastrointestinal
dysfunction
8. Myocarditis and pericarditis
9. Irritability
10. Anterior uveitis (70%)
11. Perianal erythema (70%)
Irritability
Subacute stage
(11-30 d)
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Persistent irritability, anorexia, and
conjunctival injection
Decreased temperature
Thrombocytosis
Acral desquamation
Aneurysm forms
Aneurysm forms
Convalescent/chroni
c phase (>30 d)

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Expansion of aneurysm
Possible MI
A tendency for smaller
aneurysms to resolve on their
own (60% of cases)
Diagnostic criteria
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1.Fever(> 5 days) and refractory to
appropriate antibiotic therapy
2.Polymorphous erythematous rash
3.Oropharyngeal changes, including
diffuse hyperemia, strawberry
tongue, and lip changes (eg, swelling,
fissuring, erythema, bleeding)
Diagnostic criteria
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
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4.Peripheral extremity changes,
including erythema, edema,
induration, and desquamation
5.Nonpurulent cervical
lymphadenopathy
6.Nonexudative bilateral conjunctivitis
Diagnostic criteria

Patients with classic
Kawasaki disease must
have 5 of the former
symptoms, with fever an
absolute criterion.
Differentials


Staphylococcal infection (such as
scalded skin syndrome, toxic shock
syndrome)
Streptococcal infection (such as
scarlet fever, toxic shock-like
syndrome). Throat carriage of group
A streptococcus does not exclude the
possibility of Kawasaki disease
Differentials
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Measles and other viral exanthems
Leptospirosis
Rickettsial disease
Stevens-Johnson syndrome
Drug reaction
Juvenile rheumatoid arthritis
Lab Studies
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Mild-to-moderate normochromic
anemia
moderate-to-high WBC count
ESR ↑, C-reactive protein ↑, and
serum a-1-antitrypsin ↑.
Culture results are all negative
Lab Studies
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ANA, RF,ASO normal
Platelets
• Thrombocytosis （2-3w）
• associated with severe coronary
artery disease and MI.
Liver enzymes
• AST, ALT↑
• bilirubin ↑
Lab Studies
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Cardiac enzymes ↑
（ CK，CK-MB,
cardiac troponin,
LDH）
Radiography: rule out
cardiomegaly or subclinical
pneumonitis.
Imaging Studies
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Echocardiography: rule out CAAs and
myocarditis, valvulitis, or pericardial
effusion.
Diffuse dilatation of coronary lumina can
be observed in 50% of patients by the
10th day of illness.
Echocardiography should be repeated in
the second or third week of illness and 1
month after all other laboratory results
have normalized.
Imaging Studies

Ultrasonography:
• Gall bladder
ultrasonograph
y （liver or gall
bladder
dysfunction ）
• scrotal
ultrasound to
evaluate for
epididymitis.
Imaging Studies

MRA:
•defines CAA
in patients
with
Kawasaki
disease.
•noninvasive.
Other Tests

ECG
• acute infarction.
• Tachycardia,
• a prolonged PR interval,
• ST-T wave changes,
• decreased voltage of R waves may
indicate myocarditis.
• Q waves or ST-T wave changes
may indicate an MI.
Medical Care

The main goal of treatment is to
prevent coronary artery disease and
relieve symptoms.:
Full doses of salicylates (aspirin);
intravenous gammaglobulin are the
mainstays of treatment.
Drug Category

IVIG (first line but not the sole therapy)
• Neutralizes circulating myelin antibodies
through anti-idiotypic antibodies;
• down-regulates proinflammatory cytokines,
• blocks Fc receptors on macrophages
• suppresses inducer T and B cells and
augments suppressor T cells;
• blocks complement cascade;
• promotes remyelination
IVIG
400 mg/kg/d IV as a single
daily infusion for 4 d-5d
Alternatively,;
 2 g/kg IV infused over 12 h
once as single dose

Drug Category

Aspirin
• decrease inflammation,
• inhibit platelet aggregation
• improve complications of venous
stases and thrombosis. Irreversibly
inactivates cyclooxygenase,
ultimately preventing thromboxane
A2 production in platelets.
Drug Category

Aspirin 80-100 mg/kg/d PO divided
qid for 2 wk initially; then 5-10
mg/kg PO qd for 6-8 wk until
sedimentation rate and platelet count
are within the reference range,
typically used for 6-12 wk
Drug Category

Corticosteroid
•Not recommended to use only.
Prescript only when the
therapeutic effect of IVIG is not
satisfied.
•2mg/kg, 2—4 weeks
Complications

Cardiovascular
• Significant heart failure or
myocardial dysfunction (unlikely to
occur once fever is resolved)
• Diffuse coronary artery ectasia and
aneurysm formation, giant
aneurysm (internal luminal
diameter >8 mm)
• MI
Complications

Cardiovascular
• Myocarditis (common but rarely causes
CHF)
• Valvulitis, usually mitral (only occurs in
1% of patients and rarely requires valve
replacement)
• Pericarditis with small pericardial
effusions (occurs in 25% of patients
with acute illness)
• Systemic artery aneurysms
• Rupture of CAA with hemopericardium
Other complications
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Extreme irritability, especially in
younger infants
Aseptic meningitis
Arthritis
Mild hepatic dysfunction, rarely
jaundice
巨嗜细胞活化过度综合症：死亡原因之一
Other complications
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Gallbladder hydrops (diagnosed by
means of ultrasonography but
usually resolves without surgical
intervention)
Diarrhea
Pneumonitis
Otitis media
Other complications
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Erythema and induration at the site
of BCG inoculation (reported in Japan)
Peripheral extremity gangrene
(extremely rare)
Bowel ischemia and necrosis
Prognosis
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With prompt
treatment, the
prognosis is good.
The current
mortality rate is
0.1-2%.
2% to 4%% of treated
children still develop
coronary artery disease
Thank you！