Abstract O.50: Favorable Outcome Of Patients With Kawasaki Disease Treated With Unified Protocol With Cyclosporine A As The Third Line Therapy

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Abstract

Background: Cyclosporin A (CsA), which potently suppresses inflammatory cytokines by negative regulation of nuclear factor of activated T cells pathway, may be a promising option for the treatment of Kawasaki disease (KD), especially in patients resistant to intravenous immunoglobulin (IVIG). We treat KD patients with the unified protocol using CsA after 2nd IVIG. Here we report 6 years-outcome of this treatment. Patients and Methods: From 2008 to 2014, 441 patients were treated. At the persistence or recurrence of fever at the end of the 2nd IVIG (2 g/kg for 24 hours), patients were treated with CsA (4-5mg/kg/day) through oral administration except for infants younger than 4 months old. If patients failed to become afebrile after CsA treatment, they received 3rd IVIG. All patients received aspirin 50mg/kg/day at febrile stage. Results: 419 patients became afebrile after 1st or 2nd IVIG. At this point, 2 patients had mild CALs (max 4.7 mm, 2.5mm in 2month-old infant). Among the other 22 patients (5.0%) who failed to become afebrile, one infant aged 1 month-old received 3rd IVIG. Among a total of 21 patients treated with CsA, 10 patients responded promptly to be afebrile within 5 days. The other 11 patients failed to become afebrile and received 3rd IVIG. Eight out of the 11 patients became afebrile after 3rd IVIG. Three patients developed mild CALs (max 3.1mm, 3.7mm, 5.5mm). There were no serious adverse effects in CsA treatment. A total number of patients with CAL was 5 (1.1%). The maximum CAL diameter was 5.5 mm. Conclusions: The outcome of KD patients treated with CsA and 3rd IVIG as a third line therapy is safe and favorable. CsA could be a candidate drug of the first line therapy.

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