What is Kawasaki disease (Mucocutaneous Lymph Node Syndrome)?

From the Guidelines

Kawasaki disease should be treated with intravenous immunoglobulin (IVIG) at 2 g/kg as a single infusion, along with high-dose aspirin, to reduce the risk of coronary artery aneurysms, as recommended by the American Heart Association 1. The standard treatment for Kawasaki disease involves immediate administration of IVIG and high-dose aspirin during the acute phase.

• The dosage of IVIG is typically 2 g/kg as a single infusion.
• High-dose aspirin is given at 80-100 mg/kg/day divided into four doses during the acute phase.
• After fever resolves, aspirin is reduced to a low dose of 3-5 mg/kg/day for 6-8 weeks to prevent blood clots. Key considerations in the management of Kawasaki disease include:
• Treatment should begin within 10 days of fever onset for best outcomes.
• If fever persists 36-48 hours after IVIG, a second dose may be given.
• For refractory cases, corticosteroids, infliximab, or cyclosporine might be considered. The disease typically progresses through stages including fever, rash, red eyes, swollen hands and feet, and lymph node enlargement.
• Echocardiograms are essential during diagnosis and follow-up to monitor for cardiac complications.
• Long-term cardiac follow-up may be necessary depending on the severity of coronary involvement, as the long-term prognosis is determined by the initial and current level of coronary artery involvement 1.

From the Research

Definition and Treatment of Kawasaki Disease

• Kawasaki disease is an acute systemic vasculitis that mainly affects children, characterized by symptoms such as fever, chapped lips, strawberry tongue, red eyes, rash, redness, swollen hands and feet, or skin peeling, and enlarged cervical lymph nodes 2.
• The primary treatment for Kawasaki disease is intravenous immunoglobulin (IVIG) and acetylsalicylic acid (ASA/aspirin), with doses and regimens differing between institutions 2.

Effectiveness of IVIG in Treating Kawasaki Disease

• IVIG has been shown to reduce the incidence of coronary artery abnormalities (CAAs) in children with Kawasaki disease, with a significant decrease in new CAAs at thirty days 3.
• A meta-analysis of IVIG versus placebo found a significant reduction in CAAs at thirty days, with a relative risk (RR) of 0.74 (95% CI 0.61 to 0.90) 3.
• Higher-dose regimens of IVIG have been found to reduce the incidence of CAAs compared to medium- or lower-dose regimens, with an odds ratio (OR) of 0.60 (95% CI 0.40 to 0.89) 2.

Comparison of IVIG with Other Treatments

• IVIG has been compared to ASA, with results showing that IVIG probably reduces the incidence of CAAs in people with Kawasaki disease up to 30 days, with an OR of 0.60 (95% CI 0.41 to 0.87) 2.
• IVIG has also been compared to prednisolone, with very uncertain evidence regarding the incidence of CAA, and little to no difference in adverse effects between groups 2.

Aspirin Dose in Kawasaki Disease

• The use of low-dose ASA has been associated with higher odds of IVIG resistance compared to high-dose ASA, with an OR of 3.2 (95% CI 1.1,9.1) 4.
• High-dose ASA may reduce the incidence of IVIG resistance, with no significant difference in duration of hospital stay or incidence of coronary artery aneurysms 4.

Rare Cases of Kawasaki Disease

• Kawasaki disease can occur in adults, although it is rare, and may present with cardiac involvement and acute pancreatitis 5.
• Early diagnosis and treatment with IVIG, aspirin, and corticosteroids can lead to significant clinical improvement in these cases 5.