Role of glucocorticoids in Kawasaki disease

Although treatment with intravenous immunoglobulin (IVIg) with aspirin is the standard of care for children with Kawasaki disease (KD), 15-20% of patients fail to respond and experience persistent or recurrent fever after completion of IVIg administration. These IVIg non-responders are at high risk for coronary artery lesions (CAL), and may need alternative or supplemental therapy. Based on retrospective studies (albeit with low evidence levels), glucocorticoid therapy was hitherto thought to worsen CAL. However, subsequent prospective studies have shown that prednisolone or IV methylprednisolone pulse as the initial or rescue therapy is effective in reducing CAL. A clinical trial known as the Randomized controlled trial to Assess Immunoglobulin plus Steroid Efficacy for KD (RAISE) Study, demonstrated the efficacy of prednisolone with initial IVIg, especially in predicted IVIg non-responders. Several meta-analyses have also supported the use of glucocorticoids for patients with severe forms of KD. Glucocorticoids can be considered as pre-emptive therapy for children with severe KD and as rescue therapy for initial non-responders. However, routine use of glucocorticoids in KD remains a contentious issue and would need further study.