Kawasaki Disease

Introduction

Kawasaki disease is a systemic febrile condition that predominantly affecting children <5 years old. The aetiology remains unknown, possible bacterial toxins or viral agents with genetic predisposition.

Diagnostic Criteria

Fever lasting at least 5 days PLUS at least 4 out of 5 of the following

• Bilateral non-purulent conjunctivitis
• Mucosal changes of the oropharynx (injected pharynx, red lips, dry fissured lips, strawberry tongue).
• Changes in extremities (oedema and/or erythema of the hands or feet, desquamation, beginning periungually).
• Rash (usually truncal), polymorphous but non vesicular
• Cervical lymphadenopathy

Diseases with similar presentation should be excluded.

NOTE: Clinical vigilance is needed to recognise patients with "incomplete" or "atypical" Kawasaki Disease.

Management

Early recognition and treatment are essential to reduce the risk of life-threatening complications.

• Up to 30% of untreated children develop coronary artery dilation or aneurysm.

Early (preferably within the first 10 days of the illness) administration of

• IVIG (single dose of 2 g/kg IV over 10-12 hours)
• Highly effective in preventing coronary artery complications.
• Aspirin
• Treatment dose (anti-inflammatory dose): 30-50 mg/kg/day in 3 divided doses till day 14 of illness or until patient is afebrile for 2-3 days.
• Maintenance dose (antiplatelet dose): 3-5 mg/kg (maximum dose 150 mg) PO once a day for at least 6-8 weeks or until ESR and platelet count normalise. If coronary aneurysm present, then continue aspirin until resolves.

NOTE: Owing to association with Reye's syndrome, manufacturer advises aspirin-containing preparations should not be given to children under 16 years, unless specifically indicated, e.g. for Kawasaki disease.

Refractory cases with continuing fever and other signs of inflammation ≥36 hours after completion of first IVIG dose need specialist advice for further management that may include

• Give a second dose of IVIG 2 g/kg or consider infliximab 5 mg/kg single infusion over 2 hours
• High-dose steroids (e.g. methylprednisolone)

Vaccinations

The use of immunoglobulins may impair efficacy of live-attenuated virus vaccines. Delay these vaccinations for at least 11 months.

External Links

• Paediatric Protocols for Malaysian Hospitals, 2019
  
• RCH Paediatric Handbook, 2021
  
• Treatment of Kawasaki disease by different doses of immunoglobulin: a meta analysis of efficacy and safety, 2012
  
• Late intravenous immunoglobulin treatment in patients with Kawasaki disease, 2012
  
• Indirect-comparison meta-analysis of treatment options for patients with refractory Kawasaki disease, 2019
  
• Low-dose or no aspirin administration in acute-phase Kawasaki disease: a meta-analysis and systematic review, 2021