What is the treatment for Kawasaki Disease?

Treatment of Kawasaki Disease

The standard treatment for Kawasaki Disease consists of intravenous immunoglobulin (IVIG) 2 g/kg as a single infusion given over 10-12 hours, combined with high-dose aspirin (80-100 mg/kg/day divided into four doses), which should be administered as soon as the diagnosis is established. 1

Initial Treatment Protocol

• IVIG should be administered as early as possible within the first 10 days of fever onset, as this significantly reduces 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 48-72 hours 1, 2
• 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, 2
• For children who develop coronary abnormalities, aspirin may be continued indefinitely 1
• Early treatment (within 5 days of fever onset) results in better coronary outcomes at one year compared to later treatment 3

Management of IVIG-Resistant Disease

Approximately 10-20% of patients fail to respond to initial IVIG therapy, defined as persistent or recrudescent fever 36 hours after completion of the initial IVIG infusion 1, 2. Options include:

• A second dose of IVIG (2 g/kg as a single infusion) is recommended as the first-line treatment for IVIG resistance 1, 2
• Corticosteroids should be considered for patients who remain febrile after two doses of IVIG 1
• The RAISE study protocol (intravenous prednisolone 2 mg/kg/day for 5 days followed by an oral taper) has shown efficacy in high-risk Japanese patients identified by scoring systems 1, 4
• Infliximab (a TNF-α inhibitor) has shown effectiveness in treating IVIG-resistant cases 1

Long-term Antiplatelet/Anticoagulation Management

• For patients without coronary abnormalities, low-dose aspirin should be continued until 6-8 weeks after disease onset 1, 2
• For patients with small coronary aneurysms, long-term low-dose aspirin is recommended indefinitely 2
• For patients with moderate-sized aneurysms (4-6 mm), aspirin plus a second antiplatelet agent is recommended 2
• 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 2

Special Considerations

• Measles, mumps, and varicella immunizations should be deferred for 11 months after receiving high-dose IVIG 1, 2
• Annual influenza vaccination is recommended for children on long-term aspirin therapy 1, 2
• Ibuprofen should be avoided in children taking aspirin for its antiplatelet effects as it antagonizes the irreversible platelet inhibition induced by aspirin 1

Common Pitfalls and Caveats

• Delaying treatment beyond 10 days increases the risk of coronary artery abnormalities 2
• 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 1, 2
• A moderate dose of IVIG (1 g/kg) is less effective than the standard high dose (2 g/kg) in preventing coronary artery abnormalities 5
• The highest risk for coronary artery thrombosis occurs within the first 3 months, with peak incidence in the first 15-45 days 2
• Although rare, Kawasaki disease can occur in adults and should be treated with the same IVIG protocol as in children 6