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Kawasaki disease
Dr Laurence Lacroix
16.04.2014
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DEFINITION:
o Kawasaki disease (KD) is an acute febrile
illness of childhood
o Vasculitis of medium-sized
extraparenchymal arteries
o Predilection for coronary arteries
o Leading cause of acquired heart disease
in developed countries
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Kawasaki diseaseKawasaki disease
HISTORY:
o 1st described in 1967 by a Japanese
pediatrician (Dr Tomisaku Kawasaki)
(Kawasaki T « Acute febrile mucocutaneous syndrome with lymphoid involvement with specific desquamation of the
fingers and toes in children » Jpn J Allergy (Arerugi) 1967;16:178-222)
o Mucocutaneous lymph node syndrome
o Cardiac involvement not apparent at first
o Autopsy cases: coronary artery aneurysms
and thrombosis
o KD described in children around the world
and virtually all races (Japan +++)
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Kawasaki diseaseKawasaki disease
EPIDEMIOLOGY:
o Nationwide surveys every 2 years in Japan
o Worldwide linear increase in incidence rate
since 1990’s (disease awareness and
recognition?)
o 2010: Incidence rate in Japan: 240/100’000
(0-4 y old), in the USA 20/100’000
o Heightened incidence in people of Asian
descent, both inside and outside Asia.
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EPIDEMIOLOGY:
o Peak incidence between 6 and 11 months
o 80-90% < 5 years
o Boys > Girls
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ETIOLOGY: Unknown!
Geneticsusceptibility
-Asian and Asian-
American
populations
-family members of
an index case
KD
Impaired immunoregulation
Infectious agents, toxins
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Kawasaki diseaseKawasaki disease
J Infect. 2013 Jul;67(1):1-10
ETIOLOGY:
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CLINICAL DIAGNOSIS : based on clinical criteria !
o Fever
o Usually more than 39.9°C, for at least 5 days
o High spiking and remittent
o Persists 1-2 weeks without treatment
o May not respond to antipyretics
o Usually resolves in 1-2 days after treatment with
immunoglobulins
o Altered general appearance
o Features of KD can fluctuate:
�thorough medical history to determine their presence
during the period of illness.
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Kawasaki diseaseKawasaki disease
CLASSIC CLINICAL CRITERIA(according to the American Heart Association)
o Fever of at least 5 days’ duration
o Presence of at least 4 of the following
principal features:
1. Bilateral conjunctival injection
2. Changes in lips and oral cavity
3. Cervical lymphadenopathy
4. Polymorphous exanthem
5. Changes in the extremities
o Exclusion of other diseases with similar
findings
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1. Bilateral nonexudative bulbarconjunctivitis
Cracked lips Strawberry tongue
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2. Oropharyngeal inflammation
+ diffuse erythema of the oropharyngeal mucosa
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3. Cervical lymph nodes enlargement
Unilateral (> 1.5 cm)
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4. Maculopapular rash
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5. Changes in the extremities
Erythema of palms and/or soles, edema of hands and/or feet. Hand and/or foot periungual peeling skin(week 2-3 of disease course).
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ASSOCIATED CLINICAL FEATURES:
Adapted from Circulation. 2004;110(17):2751
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Erythema and induration at BCG site
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COMPLICATIONS:
o Prognosis based on the
presence and severity of
coronary artery lesions
o Mild dilation� giant aneurysms
o 3-5% of children receiving IVIG
o up to 25% in untreated children
o More frequent in children
< 6 months and ≥5 years
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DIFFERENTIAL DIAGNOSIS:
o Viral infection
o Bacterial infection
o Drug hypersensitivity
o Stevens-Johnson syndrome
measles
adenovirus
enterovirus
Epstein-Barr virus
Scarlet fever
cervical lymphadenitis
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DIFFERENTIAL DIAGNOSIS:
o Juvenile idiopathic arthritis (systemic onset)
o Toxic shock syndrome (associated with
Staphylococcus or Streptococcus)
o Staphylococcal scalded skin syndrome (SSSS)
o Others: acrodynia (mercury toxicity)
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LABORATORY FINDINGS:
o ↑ sedimentation rate (> 40 mm/h)
o ↑ CRP
o neutrophilia with immature forms
o thrombocytosis (> 1 week, peaks at 3 weeks)
o anemia
o hypoalbuminemia
o ↑ liver enzymes
o sterile pyuria
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ECHOCARDIOGRAPHY:
o coronary artery dimensions
o myocardial function
o valve regurgitation
o pericardial effusion
Time from
diagnosis
diagnosis
echo echo echo
1-2
weeks
6
weeks
echo
12
months
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POOR CLINICAL OUTCOME RISK FACTORS:
o < 6 months, > 9 years
o Asian, Pacific Islander and Hispanic races
o Laboratory parameters:
o Neutrophilia
o Thrombocytopenia
o Hyponatremia
o Elevated CRP
o Elevated liver tests
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INCOMPLETE KAWASAKI DISEASE:
o More frequent in children < 12 m and > 5 y old
o classic features of KD but not enough to meet the
case definition.
o < 4 of the principal features but suggesting
laboratory results or echocardiography
o approximately 25% of the KD cohort
o ↑ risk of coronary artery complications (diagnostic
and treatment delay)
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4) Supplemental laboratory criteria:
-albumin ≤3.0 g/dL
-anemia for age
-elevation of ALT
-platelets after 7 days ≥450,000/mm3
-white blood cell count ≥15,000/mm3
-urine ≥10 WBC/highpower field
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COMPLEMENTARY EXAMINATION:
o Blood:
o Complete blood count (CBC):
o Anemia
o Leukocytosis
(elevated white blood cells ≥15’000/µL)
o Thrombocytosis= elevated platelets
(≥450’000/µL after 7 days of illness)
o Elevated erythrocyte sedimentation rate
o Elevated C-reactive protein
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COMPLEMENTARY EXAMINATION:
o Blood:
o AST, ALT, albumin, sodium: elevated liver function
tests, decreased albumin and natremia (capillary leak)
o Abnormal lipids
decreased levels of total cholesterol,
apolipoprotein A1 and high-density lipoprotein
o Serology: measles, EBV, ASLO
o Blood culture if suspicion of septicemia
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COMPLEMENTARY EXAMINATION:
o Urine:
Urine test strip: sterile pyuria
(presence of leukocytes with negative urine culture)
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COMPLEMENTARY EXAMINATION:
o Stools:
o Culture for enterovirus, adenovirus:
if positive, Kawasaki disease unlikely
o Search for blood may be positive
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COMPLEMENTARY EXAMINATION:
o Echocardiography:
Myocarditis
Pericardial effusion
Congestive heart failure
Valvular regurgitation (mitral> aortic)
Coronary arteries abnormalities
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COMPLEMENTARY EXAMINATION:
o Other:
o Prepare a throat swab for
o measles PCR
o Group A ß-hemolytic streptococcus culture
if positive, Kawasaki disease unlikely
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CASE DEFINITION- Major criteria:
o Fever ≥ 5 days
o Clinical criteria (signs of mucocutaneous inflammation)
1. Non-exudative bilateral conjunctivitis
2. Involvement of the lips and oral cavity:
3. erythema, cracked lips, “strawberry red” tongue, diffuse
inflammation of the oral and pharyngeal mucosa.
Usually unilateral neck lymphadenopathy (≥ 1.5 cm)
4. Polymorphic exanthem
5. Involvement of hands and feet: erythema of palms and/or
soles, edema of hands and/or feet. Hand and/or foot
periungual peeling skin(week 2-3 of disease course)
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CASE DEFINITION- Minor criteria:
o Erythrocyte sedimentation rate ≥ 40 mm/h
OR
CRP ≥ 3 mg/dL
o Echocardiography showing coronary artery or heart
involvement
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DIAGNOSTIC CERTAINTY LEVELS:
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DIAGNOSTIC CERTAINTY LEVELS:
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ACUTE PHASE TREATMENT: IVIG
Intravenous Immunoglobulins (IVIG) 2g/kg
o should be instituted less than 10 days after
fever onset
o Aim at reducing the inflammation
o 2nd dose 2g/kg if refractory disease (persistent
or recurrent fever at least 36 hours after the 1st IVIG
dose has been infused-11% to 23% of patients)
http://blogs.nejm.org/now/index.php/ivig/2
012/11/23/
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ACUTE PHASE TREATMENT: Aspirin
Aspirin (acetylsalicylic acid) 80-100 mg/kg/day
o Anti-inflammatory dosage
o divided into 4 doses/day
o until the child is afebrile
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ACUTE PHASE TREATMENT:
o In case of resistance to IVIG after a 2nd dose,
consider a
3-day course of 30 mg/kg/day intravenous
pulse of methylprednisolone
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CONVALESCENT PHASE TREATMENT:
o Aspirin (acetylsalicylic acid) 3–5 mg/kg per
day in once-daily dosing (antithrombotic
dosage)
o maintain it until the patient shows no
evidence of coronary changes by 6 to 8
weeks after the onset of illness
o For children who develop coronary
abnormalities, aspirin may be continued
indefinitely
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http://www.uptodate.com/contents/viral-meningitis
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SUGGESTED READING:
Kawasaki disease.Son MB, Newburger JW.Pediatr Rev. 2013 Apr;34(4):151-62
Diagnosis, treatment, and long-term management of Kawasaki disease: a statement for healthprofessionals from the Committee on Rheumatic Fever, Endocarditis, and Kawasaki Disease, Council on Cardiovascular Disease in the Young, American Heart Association.Newburger et al. Committee on Rheumatic Fever, Endocarditis, and Kawasaki Disease, Council on Cardiovascular Disease in the Young, American Heart Association.Pediatrics. 2004 Dec;114(6):1708-33
Kawasaki disease: part I. Diagnosis, clinical features, and pathogenesis.Bayers S, Shulman ST, Paller AS.J Am Acad Dermatol. 2013 Oct;69(4):501.e1-11; quiz 511-2. doi: 10.1016/j.jaad.2013.07.002. Review.
Kawasaki disease: part II. Complications and treatment.Bayers S, Shulman ST, Paller AS.J Am Acad Dermatol. 2013 Oct;69(4):513.e1-8; quiz 521-2. doi: 10.1016/j.jaad.2013.06.040. Review.
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QUESTIONS