Adjunctive therapies for Kawasaki disease

Abstract

Kawasaki disease (KD) is the most common cause of acquired heart disease in children in developed countries.(1,2) The primary goal of treatment is to prevent coronary artery aneurysms (CAA). Between 10 and 20% of KD patients are resistant to treatment with intravenous immunoglobulin (IVIG) and have an almost nine-fold increased risk of developing CAA.(3) In addition, approximately 80-90% of patients who go on to develop CAA have abnormal coronary artery dimensions on their first echocardiogram and can therefore be identified as high-risk patients. These two subsets of KD patients are candidates for adjunctive therapy, in addition to IVIG. Understanding the mechanism of action of IVIG may provide insight into IVIG resistance and guidance for choosing adjunctive therapies in KD. Therapeutic options in the treatment of refractory KD and patients with early CAA include additional IVIG, glucocorticoids, tumor necrosis factor inhibitors, calcineurin inhibitors and interleukin-1 (IL-1) blockers.(3-10) Animal studies suggest that the anti-inflammatory properties of statins may also be beneficial in blocking CAA progression.(6) It is unlikely that these therapies will be studied in large, randomized controlled trials in the future due to required sample size and funding constraints. Thus, data from the research laboratory may be helpful in guiding selection of the most promising adjunctive therapies.

Keywords: Adjunctive therapies; Anakinra; Atorvastatin; Cyclosporin; Glucocorticoids; Infliximab; Intravenous immunoglobulin resistance; Refractory Kawasaki disease.