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Treating Kawasaki Disease

A study published in The Lancet has shown that adding cyclosporin to intravenous immunoglobulin (IVIG) was a safe and effective way to prevent severe coronary artery outcomes in patients with Kawasaki disease who were predicted to be resistant to IVIG.

Genetic studies have suggested that the calcium-nuclear factor of activated T cells pathway may be important in the pathogenesis of Kawasaki disease, and that some patients may be resistant to IVIG therapy. This trial set out to assess whether targeting these T cells with cyclosporin (CsA) would avert coronary artery abnormalities in patients with Kawasaki disease.

This open-label, multicenter trial from Japan enrolled 175 patients with Kawasaki disease predicted to be at higher risk for IVIG resistance and randomized them to receive either IVIG plus CsA (5 mg/kg/D) for 5 days or IVIG. The primary endpoint was the incidence of coronary artery abnormalities at week 12.

The addition of CsA to IVIG yielded more favourable coronary artery outcomes in patients with Kawasaki disease who were predicted to be unresponsive to IVIG: 14% (RR 0.46, 95% CI 0.25-0.86, P=0.010) versus 31% (RR 0.46, 95% CI 0.25-0.86, P=0.010), respectively. Adverse events were not different between the groups (9% vs 7%).

Jack Cush, MD, is the director of clinical rheumatology at the Baylor Research Institute and a professor of medicine and rheumatology at Baylor University Medical Center in Dallas. He is the executive editor of A version of this article first appeared on RheumNow, a news, information and commentary site dedicated to the field of rheumatology. Register to receive their free rheumatology newsletter.