Infliximab plus plasma exchange rescue therapy in Kawasaki disease.

Abstract

To evaluate infliximab (IFX) in patients with Kawasaki disease (KD) that was unresponsive to additional intravenous immunoglobulin (IVIG) therapy and subsequent rescue with supplementary plasma exchange (PE) in patients unresponsive to treatment. We studied 76 patients with KD who received IVIG therapy twice and were unresponsive to additional IVIG. Seventy were treated with IFX alone (92.1%). Six patients who were unresponsive IFX (7.9%) were further treated by PE. This resulted in disappearance of fever and other clinical symptoms, and improvement of laboratory data. There was no severe life-threatening adverse events.Twelve of the 76 cases had developed coronary artery dilatation, and 3 had coronary artery aneurysm within 1 month of disease onset. At the end of follow-up, in all cases, coronary artery lesions were suppressed or reversed. Treatment of intractable KD with sequential IVIG, IFX, and PE treatments in a step-wise protocol was effective.