From the Guidelines
The primary treatment for Kawasaki disease is intravenous immunoglobulin (IVIG) at a dose of 2 g/kg given as a single infusion over 10-12 hours, along with high-dose aspirin (80-100 mg/kg/day divided into four doses) during the acute phase. This treatment should be initiated within the first 10 days of illness, ideally within 7 days, to reduce the risk of coronary artery aneurysms 1. After the fever resolves, aspirin is typically reduced to a low dose (3-5 mg/kg/day as a single dose) and continued for 6-8 weeks or longer if coronary abnormalities are present. For patients who remain febrile 36-48 hours after completing IVIG, a second dose of IVIG at 2 g/kg may be considered. In refractory cases, additional treatments include corticosteroids (methylprednisolone 30 mg/kg/day for 1-3 days), infliximab (5 mg/kg as a single infusion), or cyclosporine. IVIG works by modulating cytokine production and neutralizing bacterial superantigens, while aspirin provides anti-inflammatory effects and prevents platelet aggregation to reduce the risk of thrombosis. Regular cardiac follow-up with echocardiograms is essential to monitor for coronary artery complications.
Some key points to consider in the treatment of Kawasaki disease include:
- The importance of prompt initiation of treatment with IVIG and aspirin to reduce the risk of coronary artery aneurysms 1
- The use of a second dose of IVIG in patients who remain febrile after the initial treatment 1
- The potential role of additional treatments, such as corticosteroids and infliximab, in refractory cases 1
- The need for regular cardiac follow-up with echocardiograms to monitor for coronary artery complications 1
It is also important to note that the treatment of Kawasaki disease should be individualized based on the specific needs and circumstances of each patient. The American Heart Association has published guidelines for the diagnosis, treatment, and long-term management of Kawasaki disease, which provide a framework for clinicians to follow 1.
From the Research
Treatment Options for Kawasaki Disease
- The primary treatment for Kawasaki disease is intravenous immunoglobulin (IVIG) and acetylsalicylic acid (ASA/aspirin) 2, 3, 4.
- IVIG therapy results in rapid resolution of clinical symptoms in 80-90% of patients and has been shown to reduce the risk of coronary disease 2.
- The evidence to support the efficacy of aspirin in improving coronary artery outcomes is lacking 2, 5.
- High-dose IVIG regimens are probably associated with a reduced risk of coronary artery aneurysm (CAA) formation compared to ASA or medium- or low-dose IVIG regimens 3.
Management of IVIG Resistance
- Patients who fail to respond to the first dose of IVIG will most often receive a second dose 2.
- Patients who fail to respond to two doses of IVIG present a unique challenge, and treatment with infliximab, cyclosporine, or methotrexate may be considered 2.
- The role of steroids in high-risk non-Japanese patients is unclear, with the biggest challenge being early identification of patients at high risk of developing adverse coronary artery outcomes 2.
Aspirin Dose in Kawasaki Disease
- The use of high-dose aspirin in the treatment of Kawasaki disease is controversial, and some studies suggest that it may not have a significant role in preventing coronary artery abnormalities 6, 5.
- Low-dose aspirin was associated with higher odds of IVIG resistance compared to high-dose aspirin, with no significant difference in duration of hospital stay or incidence of coronary artery aneurysms 6.
- Eliminating high-dose aspirin in the treatment of Kawasaki disease did not increase the risk of coronary artery abnormalities 5.