Corticosteroid / Immunoglobulin Combination May Benefit Patients With High-Risk Kawasaki Disease

Nurse holds blood sample to treat Sars-CoV-2-related Kawasaki disease in children under five, conceptual image, unbranded generic drug containers and hypothetical bar codes
Corticosteroids combined with immunoglobulin treatment may be beneficial for some patients at high risk for Kawasaki disease.

Corticosteroids combined with immunoglobulin treatment may be beneficial for some patients at high risk for Kawasaki disease (KD), according to a retrospective cohort study published in the Journal of the American Heart Association.

In this study, data from 115 hospitals and 1000 unique sets from the 2009 to 2010 and 2015 to 2016) nationwide KD surveys in Japan were analyzed. The nationwide surveys compared the efficacy of standard treatment (2 g/kg intravenous immunoglobulin therapy infused with aspirin) alone or in combination with corticosteroids (n=1593 in each group) for the treatment of KD. The 2 groups were balanced for balanced for demographic characteristics and recurrence status. Participants were assessed for coronary artery abnormalities (CAAs).

There was a general trend in this cohort for an increased use of combination therapy and a decrease in CAAs. In total, 7.1% and 5.4% of patients in the first and second surveys, respectively had a CAA. Treatment failure increased from 18.6% to 19.1% from the earlier to the later survey.

A median of 44.3% of patients received the combination treatment (95% CI, 42.1%-46.5%), and there was a median of 4.6% and 8.8% of patients who developed CAAs in the treatment and control groups, respectively (95% CI, 3.8%-5.8% and 95% CI, 7.5%-10.0%, respectively; risk ratio,  0.53; 95% CI, 0.41-0.67).

Treatment failure occurred at a greater rate in the treatment vs control group (14.1%; 95% CI, 12.4%-15.9% vs 21.7%; 95% CI, 19.8%-23.4%, respectively; risk ratio, 0.65; 95% CI, 0.56-0.75). The risk ratios in patients considered to be at low risk were 0.98 for CAAs (95% CI, 0.88-1.11) and 1.08for treatment failure (95% CI, 1.02-1.15).

A major limitation of this study was that information on the type, dose, and duration of corticosteroid treatment was not included in the surveys.

“Our study suggested a substantial protective effect of combination treatment with multiple-dose corticosteroids in a large sample of patients obtained from Japanese nationwide KD surveys,” noted the study authors. “Significant reductions in CAAs and treatment failure were observed among groups of patients with KD with high usage of combination treatment. Our analyses provide evidence that combination treatment with multiple-dose corticosteroid may be effective in preventing CAAs for selected patients at high risk for KD.”


Ae R, Abrams J Y, Maddox R A, et al. Corticosteroids added to initial intravenous immunoglobulin treatment for the prevention of coronary artery abnormalities in high-risk patients with Kawasaki disease. J Am Heart Assoc. 2020;9(17):e015308. doi:10.1161/JAHA.119.015308