Kawasaki disease

Background

  • Also known as: mucocutaneous lymph node syndrome
  • Vasculitis of unknown etiology
    • Likely a combination of genetic, infectious, and regional factors
  • Peaks at 18-24 months
    • Rare in <4mo, >5yr
  • Leading cause of acquired heart disease in children
  • Coronary aneurysm more common in incomplete than in classic KD
  • MIS-C has a number of overlapping clinical features, but appears to be a separate diagnosis at this time.

Vasculitis Syndrome Types

Clinical Features

Strawberry tongue.
Kawasaki disease signs
(a) Strawberry tongue; (b) Conjunctival injection; (c) Periungual desquamation.
  • Fever that is high, abrupt. Anyone with a fever >5 days should be considered for a Kawasaki workup.
  • Rash accompanies onset of fever
    • Often seen in perineum. Presents as erythema and desquamation
    • Later there are macular and morbilliform lesions on the trunk, arms, and legs
    • No vescicles or bullae
    • Maculopapular most common; vesicles not seen
  • Conjunctivitis
    • Bilateral and non-exudative in 90% of patients
    • Anterior uveitis with cell and flare in 70% of patients but is more specific and suggestive of KD
  • Mucositis: Cracked lips and strawberry tongue. Vesicles or ulcers suggest a pathology other than KD
  • Edema of the hands and feet
    • Desquamation around the nails in ~90%
  • Lymphadenopathy is the least consistent finding in 50-75% of children
    • Usually anterior cervical
    • Less common in those under one year of age
  • Cardiac complications develop early on
    • Coronary artery aneurysm development is most prevalent as fever lessens
  • KD shock syndrome (7%)

Associated Symptoms

Differential Diagnosis

Pediatric fever

Erythematous rash

Evaluation

Work-Up

  • CBC
  • Labs
  • LFTs- elevated
  • ESR, CRP- elevated
  • Blood culture
  • Urinalysis
  • ECG
  • Viral panel: A positive viral PCR does not exclude KD
    • 42% of children with KD had a +viral respiratory panel on initial presentation
  • TTE (Coronaries, LV, valves)
    • Consider if f ESR≥ 40, CRP≥ 3, or if desquamation occurs
  • Red Top "Kawasaki Serum to CBR"

Evaluation

  • Clinical diagnosis
Classic Kawasaki Disease Fever for 5 days or more plus four of the following symptoms
1. Bilateral nonexudative conjunctivitis
2. Mucous membrane changes (erythema, peeling, cracking of lips, "strawberry tongue," or diffuse oropharyngeal mucosal erythema)
3. Changes of the extremities (erythema or swelling of hands/feet, peeling of finger tips/toes in the convalescent stage)
4. Rash
5. Cervical adenopathy (more than one node >1.5 cm unusually unilateral anterior cervical)
Incomplete Kawasaki Disease Fever for 5 days and two to three clinical criteria of classic Kawasaki disease plus
C-reactive protein 3.0 milligrams/L and/or erythrocyte sedimentation rate 40 mm/h plus positive echocardiogram or three or more of the following:
1. Albumin <3 grams/dL
2. Anemia
3. Elevated alanine aminotransferase (ALT)
4. Platelets >450,000/mm3 7d after fever onset
5. White blood cell count >12,000/mm3
6. Pyuria

Management

Assess risk for IVIG resistance (There are 4 scoring systems):

Scoring System Nation Enrolled patients IVIG resistance Sensitivity Specificity Risk Factors Point Predicted risk (score)
Egami Japan 320 41 78 76 Age <6 mo 1 Low risk (0-2)
Illness <4 days 1
CRP 8+ mg/dL 1 High risk (3+)
ALT 80+ mg/dL 2
Platelets < 300 1
Kobayashi Japan 528 148 86 68 Age <12 mo 1 Low risk (0-3)
Illness days <4 days 2
CRP >10 mg/dL 1 High risk (4+)
ALT >100 mg/dL 2
Platelets < 300 1
Na <133 2
Neutrophils >80% 2
San Diego US 362 60 73.3 61.9 Illness <4 days 1 Low risk (0-1)
GGT 60+ IU/L 1
zHgb < -2 1 High risk (2+)
Bands >20 2
Formosa Taiwan 248 29 86.2 81.3 Lymphadenopathy 1 Low risk (0-2)
Neutrophils >60% 2 High risk (3+)
Albumin <3.5g/dL 1
  • IVIG 2gm/kg over 12hr
    • Requires inpatient monitoring for allergic reaction
    • May increase ESR as IGs are positively charged which increases RBC sedimentation[1]
  • Aspirin
    • 30-100mg/kg/day divided 4 times a day is often used
    • Has not been shown to prevent coronary artery aneurysms
    • 3-5mg/kg/day is appropriate for anti-platelet activity
    • Reye Syndrome has not been reported in association with KD treatment with aspirin
  • Steroids
    • For high-risk patients
    • Decreases CAA formation if started with IVIG
    • Using it as a rescue for IVIG-resistant patients is less effective

Disposition

  • Admit
  • Follow-up cardiac evaluation for coronary aneurysm screening

See Also

External Links

References

1. PMID: 24678327

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