Treatment for Kawasaki Disease
The standard treatment for Kawasaki disease consists of intravenous immunoglobulin (IVIG) at 2 g/kg as a single infusion plus aspirin, which should be administered as soon as the diagnosis is established, ideally within the first 10 days of illness. 1
First-Line Treatment Protocol
IVIG Administration
- Dose: 2 g/kg as a single infusion
- Timing: As soon as diagnosis is established, ideally within first 10 days of illness
- Administration: Usually given over 10-12 hours
- Efficacy: Reduces risk of coronary artery abnormalities from 20-25% to less than 5% 1
Aspirin Therapy (Two-Phase Approach)
Acute Phase:
- High-dose aspirin: 80-100 mg/kg/day divided into four doses
- Continue until patient is afebrile for 48-72 hours 1
Convalescent Phase:
- Low-dose aspirin: 3-5 mg/kg/day as a single dose
- Continue until 6-8 weeks after disease onset if no coronary abnormalities develop 1
Management of IVIG Resistance
Approximately 10-20% of patients develop recrudescent or persistent fever at least 36 hours after IVIG infusion (IVIG resistance) 1. For these patients:
- Administer a second IVIG dose of 2 g/kg if fever persists or recurs within 36 hours after initial IVIG 1
Follow-up and Monitoring
Echocardiography Schedule
- At diagnosis
- Within 1-2 weeks after treatment
- 4-6 weeks after treatment for uncomplicated cases
- More frequent monitoring for patients with coronary abnormalities 1
Long-term Management
- Long-term aspirin therapy for patients who develop coronary artery abnormalities
- Annual influenza vaccination for children on long-term aspirin therapy (to reduce risk of Reye syndrome) 1
- Ongoing cardiac monitoring based on degree of coronary involvement
Important Clinical Considerations
High-Dose vs. Low-Dose Aspirin
Recent research has shown conflicting results regarding aspirin dosing:
- Some studies suggest high-dose aspirin may be associated with lower rates of IVIG resistance compared to low-dose aspirin 2
- Other studies have found no benefit of high-dose over low-dose aspirin in preventing coronary artery abnormalities 3
Emerging Treatments
- Adjunctive corticosteroid treatment is being investigated as a potential addition to standard therapy to reduce coronary artery aneurysm rates 4
- For cases resistant to multiple IVIG treatments and steroids, methotrexate has been reported as a potential treatment option in isolated cases 5
Diagnostic Reminders
For accurate diagnosis and timely treatment, remember:
- Kawasaki disease requires fever persisting for at least 5 days plus at least 4 of 5 principal clinical features
- In patients with ≥4 principal clinical criteria, diagnosis may be made with only 4 days of fever
- Consider incomplete Kawasaki disease in children with prolonged unexplained fever and fewer than 4 principal clinical findings 1
Common Pitfalls to Avoid
- Delayed diagnosis, particularly in infants under 6 months who may present with prolonged fever and irritability as the only clinical manifestations
- Failure to initiate treatment promptly, which increases risk of coronary artery abnormalities
- Not considering IVIG resistance when fever persists or recurs after initial treatment
- Inadequate follow-up echocardiography to monitor for coronary involvement
Early recognition and prompt treatment are critical to reduce the risk of coronary artery abnormalities, which represent the most serious complication of Kawasaki disease.