What is the current treatment regimen for Kawasaki disease?

Current Treatment Regimen for Kawasaki Disease

The current standard treatment for Kawasaki disease consists of a single infusion of intravenous immunoglobulin (IVIG) at 2 g/kg, combined with aspirin therapy, which should be administered within the first 10 days of illness and preferably within 7 days of onset. 1

Initial Treatment Protocol

Primary Therapy

• IVIG Administration:
  • Dose: 2 g/kg as a single infusion
  • Timing: Within 10 days of fever onset when possible 1
  • For patients beyond day 10, treatment should still be given if there are clinical and laboratory signs of ongoing inflammation 1

Aspirin Therapy

• High-dose aspirin phase:

  • Dose: 80-100 mg/kg/day divided into four doses
  • Duration: Continue until patient is afebrile for 48-72 hours 1

• Low-dose aspirin phase:

  • Dose: 3-5 mg/kg/day for antiplatelet effect
  • Duration: Continue for 6-8 weeks if no coronary abnormalities develop 1
  • For patients who develop coronary artery abnormalities, long-term aspirin therapy is recommended 1

Management of Treatment Resistance

IVIG Resistance

• Consider a second IVIG dose of 2 g/kg if fever persists or recurs within 36 hours after initial IVIG 1
• Consider corticosteroid therapy or infliximab for patients who remain resistant to IVIG 1

Role of Corticosteroids

• While historically controversial, more recent evidence suggests potential benefit of corticosteroids in certain cases
• Corticosteroids may be particularly useful for IVIG-resistant cases 2
• Some studies have shown that adding methylprednisolone to conventional therapy resulted in shorter fever duration and hospital stays 2

Monitoring and Follow-up

Cardiac Monitoring

• For treated patients without coronary abnormalities:

  • Echocardiography at diagnosis
  • Repeat within 1-2 weeks
  • Final echo at 4-6 weeks after treatment 1

• For patients with coronary abnormalities:

  • More frequent echocardiography
  • Consider additional cardiac imaging modalities 1

Special Considerations

High-Risk Patients

• Infants <6 months are at particularly high risk of developing coronary artery abnormalities
• These infants often present with prolonged fever as the sole clinical finding
• Require a lower threshold for evaluation and treatment 1

Aspirin Dose Controversy

• While the American Heart Association recommends high-dose aspirin initially, there is ongoing debate about optimal dosing
• Some research suggests high-dose aspirin may reduce IVIG resistance compared to low-dose aspirin 3
• Annual influenza vaccination is recommended for children on long-term aspirin therapy to reduce the risk of Reye syndrome 1

Timing of Treatment

• Treatment before day 5 of illness appears no more likely to prevent cardiac sequelae than treatment on days 5-7
• However, early treatment may be associated with an increased need for IVIG retreatment 2

Efficacy and Outcomes

• Even with optimal treatment within the first 10 days of illness, approximately 5% of children develop at least transient coronary artery dilation and 1% develop giant aneurysms 2
• Lower IgG levels have been associated with worse outcomes, supporting the relationship between serum IgG concentration and therapeutic effectiveness 2

Vaccination Considerations

• Measles and varicella immunizations should be deferred for 11 months after high-dose IVIG administration 2
• If risk of measles exposure is high, vaccination may be given earlier with reimmunization 11 months after IVIG if serological response is inadequate 2

The evidence clearly demonstrates that prompt treatment with IVIG and aspirin significantly reduces the risk of coronary artery abnormalities, which is the most serious complication of Kawasaki disease. Clinicians should maintain a high index of suspicion for Kawasaki disease in children with prolonged unexplained fever, particularly in infants who may present with incomplete clinical criteria.