Kawasaki
Disease
Morning Report
July 16th, 2013
Epidemiology
• Previously known as mucocutaneous lymph node
syndrome
• Incidence is greatest in children in East Asia or are
of Asian ancestry
• Boys more commonly affected than girls
• 80-90% of cases in children < 5 yo
• Most countries noted increase in cases since early
2000s
• Risk factors for poor coronary artery outcomes:
o Young age, especially < 6 months
o Asian and Pacific Islander race
o Hispanic ethnicity
Pathogenesis
• Unknown!
• Infectious?
o Seasonal peaks occurred in US and Japan w/ increased
incidence in localized areaspossible transmissable vector
o Toxin-mediated? Rash can resemble erythroderma similar
to TSS or SSSS and IVIG could work by binding toxin
• Infection-triggered Inflammation
o Many patients w/ KD have documented concomitant
infections
o Infectious agents could trigger an inflammatory cascade
Clinical Manifestations
• Diagnosis requires fever > 5 days and at least 4 of the
following features:
Conjunctivitis
Rash
Adenopathy
Strawberry Tongue
Hands/Feet
and
Burn
Clinical Manifestations
• Fever
o Must be present ≥ 5 days
o Most consistent manifestation
o Minimally responsive to anti-pyretics
o Remains > 38.5 during most of illness
Clinical Manifestations
• Conjunctivitis
o Bilateral non-exudative
o Present in >90% of
patients
o Mainly bulbar injection
which spares the limbus
o Can be associated w/
photophobia
o Typically begins within
days of the onset of fever
o Anterior uveitis may also
be seen on slit lamp exam
Clinical Manifesations
• Rash
o Usually appears within 5
days of fever onset
o Often starts as
desquamation in
perineal area and
evolves into a diffuse,
erythematous
maculopapular rash
o Morbilliform and
targetoid rashes can
also occur
o Vesicular/bullous lesions
are rare
Clinical Manifestations
•Adenopathy
o Unilateral
o Located in anterior cervical chain
o Non-fluctuant and non-tender
o Diameter should be > 1.5 cm
o Cervical lymph node enlargement is
least consistent feature
Clinical Manifestations
• Strawberry Tongue (Mucositis)
o Cracked, red lips
o Strawberry tongue (sloughing of
filiform papillae and denuding of
inflamed tissue)
o Discrete oral lesions are NOT seen
Clinical Manifestations
• Hand/Feet
Changes
o Indurated edema of the
dorsum of their hands and
feet
o Diffuse erythema of palms
and soles
o Characteristic periungual
peeling from fingers and
toes begins 2-3 weeks after
the onset of fever
o Generally the last
manifestation to appear
Other Manifestations
• Arthritis
o Up to 25% of patients
o Large joints primarily involved
• GI (61%)
o
o
o
o
Diarrhea
Vomiting
Abdominal Pain
Hydrops of the gallbladder
• Irritability (50%)
o Likely due to meningeal inflammation
• Cough or Rhinorrhea (35%)
Lab Findings
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•
•
•
•
•
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Elevated Neutrophil count w/ bandemia
Elevated ESR and CRP
Anemia (normocytic, normochromic)
Hypoalbuminemia
Thrombocytosis (after the 1st week of illness)
Sterile pyuria
Elevated serum transaminases (40%)
Abnormal plasma lipids
Pleocytosis of CSF
• Echo: evaluate for coronary aneurysms, decreased LV
functioning, pericardial effusions
Management
• Echo
o Obtained at diagnosis, 1-2 weeks later, and 6 weeks post-discharge
• IVIG
o 2g/kg
o Ideally, given within first 7 days of illness, and by day 10 (from 1st day of
fever)
o 15% will have persistent fever after 1st doseresistant to IVIG
• Controversial, but most are given another dose after day 10 if
persistent fever
• Aspirin
o High dose (80-100 mg/kg/d): Anti-inflammatory, given until afebrile x 48h
o Low dose (3-5mg/kg/d): Anti-thrombotic, given until echo results are
normal after 6 weeks
Prognosis
• Relates entirely to extent of
cardiac involvement
• If IVIG given in time,
incidence of coronary artery
lesions is 5%
o Aneurysms can regress to normal lumen
diameter, but endothelial function is still
impaired in these segments
o Stenoses can developincreased risk
of myocardial ischemia
• Most children do well after
single dose of IVIG
• Mortality is < 0.5%
o Highest risk is in the first year after onset
of illness because of acute MI in
patients with giant aneurysms