From the Guidelines
The recommended initial treatment for Kawasaki disease is high-dose intravenous immunoglobulin (IVIG) at 2 g/kg as a single infusion, combined with high-dose aspirin (80-100 mg/kg/day divided into four doses). This treatment should be initiated as soon as possible after diagnosis, ideally within the first 10 days of illness 1. The use of IVIG has been shown to reduce the risk of coronary artery abnormalities from about 25% to less than 5% 1.
Key Considerations
- The treatment should be started as soon as possible after diagnosis, ideally within the first 10 days of illness, to maximize the effectiveness of IVIG in reducing coronary artery abnormalities 1.
- Aspirin is used in conjunction with IVIG for its anti-inflammatory and anti-platelet effects, with the dose adjusted based on the patient's response and the presence of coronary artery abnormalities 1.
- For patients who remain febrile 36-48 hours after the initial IVIG treatment, a second dose of IVIG may be considered, and in refractory cases, additional treatments such as corticosteroids, infliximab, cyclosporine, or other immunomodulators might be necessary 1.
Long-term Management
The long-term management of Kawasaki disease involves monitoring for coronary artery abnormalities and adjusting treatment accordingly. Echocardiography is a crucial tool in this monitoring process, allowing for the early detection of aneurysms and other abnormalities 1. The decision to continue or adjust treatment, including the use of anti-platelet therapy, should be made in consultation with specialists experienced in managing Kawasaki disease.
Evidence Quality and Recommendations
The recommendations provided are based on the highest quality evidence available, including guidelines from the American Heart Association 1. While the evidence supports the use of IVIG and aspirin as the initial treatment for Kawasaki disease, the management of refractory cases and the long-term care of patients with coronary artery abnormalities require careful consideration and consultation with specialists.
From the Research
Kawasaki Disease Management
The recommended initial treatment for Kawasaki disease management includes:
- Intravenous immunoglobulin (IVIG) and aspirin as standard initial therapy 2
- The use of corticosteroids in addition to IVIG and aspirin may improve outcomes in patients, particularly in those at high risk of developing coronary artery aneurysms 3
- A moderate dose (1 g/kg) of IVIG may be used, but the efficacy in preventing coronary artery abnormalities is lower than that of the high dose regimen (2 g/kg) 4
Treatment Options
Some key points to consider:
- IVIG therapy results in rapid resolution of clinical symptoms in 80-90% of patients and reduces the risk of coronary disease 2
- The role of aspirin in improving coronary artery outcomes is unclear, but it remains a standard part of the initial management of Kawasaki disease 2
- The optimal dose of aspirin is still a topic of debate, with ongoing trials aiming to determine the efficacy of low-dose versus moderate-dose aspirin in reducing the risk of coronary artery abnormalities 5
- Patients who do not respond to initial therapy may require additional treatment, including a second dose of IVIG or the use of other immunosuppressive agents 2, 6
High-Risk Patients
For patients at high risk of developing coronary artery aneurysms, such as those with IVIG resistance, young infants, men, highly inflamed patients, and/or those with coronary changes at diagnosis: