Treatment of Kawasaki Disease
Initial Treatment Protocol
- The American Academy of Pediatrics recommends intravenous immunoglobulin (IVIG) at a dose of 2 g/kg as a single infusion, combined with high-dose aspirin (80-100 mg/kg/day divided into four doses), administered as early as possible within the first 10 days of fever onset, to significantly reduce the risk of coronary artery abnormalities 1
- IVIG should be administered promptly after diagnosis to reduce the risk of coronary artery abnormalities 1
- High-dose aspirin (80-100 mg/kg/day divided into four doses) should be given concurrently with IVIG and continued until the patient is afebrile for at least 48 hours 1
- After fever resolution, aspirin should be reduced to low-dose (3-5 mg/kg/day as a single daily dose) and continued until 6-8 weeks after disease onset if no coronary abnormalities are present 1
Management of IVIG-Resistant Disease
- A second dose of IVIG (2 g/kg as a single infusion) is the first-line treatment for IVIG resistance, as recommended by the American Heart Association 2, 1
- High-dose pulse steroids (methylprednisolone 20-30 mg/kg intravenously for 3 days) are an alternative to second IVIG infusion 2
- Infliximab (5 mg/kg) is an alternative to second IVIG or corticosteroids for IVIG-resistant patients 2, 1
Long-term Antiplatelet/Anticoagulation Management
- For patients without coronary abnormalities, low-dose aspirin (3-5 mg/kg/day) should be continued until 6-8 weeks after disease onset, as recommended by the American Academy of Pediatrics 1
- For patients with giant aneurysms (≥8 mm), low-dose aspirin plus warfarin (target INR 2.0-3.0) or aspirin plus therapeutic doses of low-molecular-weight heparin is recommended 1
Monitoring
- Frequent echocardiography and ECG evaluation during the first 3 months after diagnosis are recommended, especially for patients with giant coronary aneurysms, according to the American Heart Association 1
- The highest risk for coronary artery thrombosis occurs within the first 3 months, with peak incidence in the first 15-45 days 1
Special Considerations and Caveats
- Incomplete Kawasaki disease (fever plus fewer than 4 classic criteria) should still be treated if there is evidence of coronary artery abnormalities or elevated inflammatory markers, as recommended by the American Academy of Pediatrics 1
- Measles and varicella immunizations should be deferred for 11 months after high-dose IVIG administration, according to the Centers for Disease Control and Prevention 1
- Annual influenza vaccination is recommended for children on long-term aspirin therapy, as recommended by the American Academy of Pediatrics 1