From the Guidelines
The primary treatment for Kawasaki disease is intravenous immunoglobulin (IVIG) and aspirin, with IVIG administered as a single infusion of 2g/kg over 10-12 hours within the first 10 days of fever onset, as recommended by the American Heart Association 1. The treatment approach for Kawasaki disease is focused on reducing inflammation and preventing complications, particularly coronary artery aneurysms.
- High-dose aspirin (80-100mg/kg/day divided into four doses) is given during the acute phase while the patient is febrile,
- then reduced to low-dose aspirin (3-5mg/kg/day as a single dose) once fever resolves. Low-dose aspirin is typically continued for 6-8 weeks if no coronary artery abnormalities develop, or longer if coronary artery abnormalities are present. For patients who don't respond to initial IVIG treatment (approximately 10-20%), a second dose of IVIG may be given, as suggested by studies 1. Some cases may require additional treatments such as corticosteroids, infliximab, or cyclosporine for refractory disease, with corticosteroids potentially being beneficial in reducing fever and coronary artery abnormalities in certain cases 1. Prompt treatment is crucial as it significantly reduces the risk of coronary artery aneurysms, which are the most serious complication of Kawasaki disease, with the risk reduced from 25% to approximately 4% with timely IVIG treatment 1. The medications work by reducing inflammation throughout the body, particularly in the blood vessels, helping to prevent damage to the coronary arteries that supply blood to the heart. Long-term antithrombotic management may be necessary for patients with persistent coronary artery abnormalities, tailored to the degree of coronary artery involvement 1.
From the Research
Treatment for Kawasaki Disease
The treatment for Kawasaki disease typically involves the use of intravenous immunoglobulin (IVIG) and aspirin.
- IVIG is administered to reduce the risk of coronary artery abnormalities, with a standard dose of 2 g/kg being strongly effective in reducing this risk 2.
- Aspirin is often given in conjunction with IVIG, although the evidence base for its use is less secure 2.
IVIG Dosage and Efficacy
- A moderate dose of IVIG (1 g/kg) has been shown to be less effective in preventing coronary artery abnormalities compared to a high dose regimen (2 g/kg) 3.
- The efficacy of IVIG in preventing coronary artery abnormalities is influenced by factors such as the duration of fever before starting IVIG and the patient's hemoglobin level 3.
Role of Aspirin in Treatment
- The use of high-dose aspirin in the treatment of Kawasaki disease has been questioned, with some studies suggesting that it may not have a significant role in preventing coronary artery abnormalities 4.
- A randomized controlled trial found that IVIG alone was as effective as IVIG combined with high-dose aspirin in preventing coronary artery lesions 5.
- Another study found that low-dose aspirin was associated with higher odds of IVIG resistance compared to high-dose aspirin, although there was no significant difference in the incidence of coronary artery aneurysms 6.
Management of Refractory Kawasaki Disease
- Approximately 10-30% of patients with Kawasaki disease do not respond to initial IVIG treatment and are at increased risk of coronary artery aneurysms 2.
- Management options for IVIG-nonresponsive patients include further doses of IVIG, corticosteroids, TNF-α blockade, cyclosporin A, anti-IL-1, and anti-CD20 therapy 2.