Kawasaki Disease

Department of Pediatrics
Purpose and Requirement

Understand the etiology and pathology of Kawasaki

Disease (K D)
Master the clinical manifestations and diagnostic
criterion of KD.
Master the treatment of KD.
 Outline
Kawasaki disease formerly known as
mucocutaneous lymphnode syndromeMCLS. 80%
of patients are younger than 5yr, Approximately 20% of
untreated patients develop coronary artery
abnormalities including aneurysms.
kawasaki disease has replaced acute rheumatic fever
as the leading cause of acquired heart disease in children
in our country.
 Outline
May lead to ischemic heart disease or
sudden death

First described in Japan in 1967 by

Dr.Kawasaki
 Outline
More common in winter and early
spring months

Occurring in both endemic and

community-wide epidemic forms
 Outline
Higher rates of KD in the siblings and
twins suggest a possible role for
genetic predisposition that interacts
with exposure to the etiologic agent or
agents in the environments
 Outline
15%-25% Coronary artery aneurysms
or ectasia develop in 15%-25% of
untreated cases
In-hospital mortality rate is about 0.17%

Recurrence rate is reported to be 1-3%

 Etiology
The etiology of KD remains unknown

Clinical and epidemiological features

suggest infection is etiology or trigger
 Etiology
Bacterial toxin: There is a hypothesis
that KD is related with superantigen

It is possible that KD results from an

immunologic response triggered by
some microbial agents
 Pathology
Generalised systemic vasculitis
involving blood vessels throughout
body.

Aneurysms occurring in arteries

Arteial remodeling Stenosis

CLINICAL MANIFESTATION

Fever
Rash of various forms
Mucosa vary
Cervical lymphnode swelling
Changes of the peripheral extremities
Rash of various forms

Rash involving
the trunk and
the extremites
Conjunctival hyperemia
Swelling of the hands
Swelling of the feet
Hard edema
Membrane peeling
Periungual desquamation tips of the fingers and toes
Strawberry tongue, dry cracked lips erythema
Strawberry tongue
Lips chapped
 Conjunctivitis
erythema of the oral and pharyngeal mucosa
BCG vaccination erythema
Erythema multiforme skin
 The erythematous rash or
maculopapular eruption is common
,mainly with involvement of the trunk
Cervical lymph
node swelling

Usually
unilateral
with a
node size
of 1.5cm
or greater
in diameter
Coronary artery aneurysm
 Other clinical findings
Cardiovascular system: myocarditis,
pericarditis, arrythmia, heart failure

Gastrointestinal tract: abdominal pain,

diarrhea, hepatic dysfunction

Genitourinary system: urethritis

 Other clinical findings
Central nervous system: extreme
irritability, aseptic meningitis

Musculoskeletal system: Arthritis,

arthralgia
 Laboratory findings
Leukocytosis with neutrophilia
Elevated erythrocyte sedimentation
rate (ESR)
Elevated C- reactive protein (CRP)
Thrombocytosis
 Laboratory findings
Anemia, Hypoalbuminemia
Elevated serum transaminases
Elevated serum IgG,IgM,IgA,IgE and
CIC
 Laboratory findings
Electrocardiogram
Sinus tachycardia, elevated S-T
segment
Echocardiogram
Pericardial effusion, valvular
regurgitation
 Two-dimensional echocardiography
is the most useful test to monitor
the potential development of
coronary abnormalities
Diagnosis criteria
Fever lasting for at least 5 days
Presence of at lease four of the following five signs:
Bilateral bulbar conjunctival injection,
generally nonpurulent
changes in the mucosa of the oropharynx ,
including injected pharynx,injected and/or dry
fissured lips, strawberry tongue
Diagnosis criteria
Changes of the peripheral extremities,such as
edema and/or erythema of the hands or feet in the
acute phase; or periungual desquamation in the
subacute phase
Rash, primarily truncal;polymorphous but
nonvesicular
Cervical adenopathy, 1.5cm ,usually unilateral
lymphadenopathy
Illness not explained by other known disease
process
 Diagnosis criteria

essential conditionUnexplained fever lasts at least 5 days or

more

Other: should meet the following four of five signs

The non-exudative conjunctival congestion
Oral pharyngeal change
Changes in limb,
Pleomorphic rash
Acute phase non suppurative cervical lymph node was
swollen
 Diagnosis criteria
If less than the above four clinical signs,
but there are clear coronary artery
lesions, can still be diagnosed with
kawasaki disease
 differentiation

1.scarlatina
 Treatment
. Anti-inflammation
1.Aspirin 30-100mg/kg.d qid)
With activities of anti-inflammation and
anti-platelet
Not lowering the frequency of the
development coronary abnormalities
Duration of Aspirin administration

redused to 3-5mg/kg.d after 14 days of

illness and the child has been afebrile
for 48-72 hrs
a.maintained for 6-8 weeks if no
coronary abnormalities detacted.
b.coronary abnormalities :Continued
indefinitely till recover.
 High doses of intravenous
immune globulin (IVIG)

2g/kg.d in a single infusion for 8-12hrs

Together with aspirin
Started within first 10 days of illness.
(If possible, within 7 days of illness)
 Corticosteroids

Not used for the initial treatment of KD

The effects on coronary artery
abnormalities still uncertain
Used to combind with IVIG and aspirin
or To the cases failed to respond to
initial therapy
 .Anti-platelet and
anticoagulant therapy
Dipyridamole (Persantin) 35mg/kgd

Warfarin
Low-molecular-weight heparin
.Symptomatic treatment
 Risk factors for coronary
aneurysms
Male sex
Age <6 months or >3 yrs
Fever more than 2 wks
ESR>100mm/h
Hemoglobin<80g/L
WBC>16x109/L
 Long-term follow-up
1m,3m,6m,1y
every 6m