Continuous Infusion of Cyclosporin A in Intravenous Immunoglobulin Resistant Kawasaki Disease Patients

Treatment of intravenous immunoglobulin (IVIG) resistant Kawasaki disease (KD) is challenging. Continuous infusion of cyclosporin A (CICyA) was employed for 4 patients with IVIG-resistant KD: these patients had remained febrile or had had recurrence of fever within 24 hours after the initial 2g/kg followed by additional 2g/kg IVIG. Response differed between initial doses of CICyA, 1.5 mg/kg/day vs. 3.0 mg/kg/day. The clinical course of the two patients treated with 1.5 mg/kg/day CICyA suggested that the low serum concentration by gradual increase of CICyA did not improve fever or inflammation. The other two patients treated with 3.0 mg/kg/day CICyA became afebrile within 24 hours after initiation of CICyA. They had a subsequently reduced dose of cyclosporin A (CyA) to maintain its appropriate serum concentration (300-500 ng/ml). No coronary artery lesion was detected one month after the onset of KD. The initial dose of 3 mg/kg/day was effective without major side effects and would be a treatment option for IVIG-resistant KD. (