Abstract 15099: Inflammatory Cytokine Profiles Under Cyclosporin Treatment for Refractory Kawasaki Disease
(Background) Kawasaki disease (KD) is an acute systemic vasculitis occurring in medium-sized arteries, especially coronary arteries. Patients with KD who fail to respond to standard therapy with intravenous immunoglobulin (IVIG) have higher risk to develop coronary artery lesions. Cyclosporin A (CyA) is one option for refractory KD, however, the mechanism how it suppresses inflammation of KD is unknown.
(Methods and Results) We analyzed time-line profiles of multiple cytokines (IL-1b, IL-2, sIL-2R, IL-4, IL-5, IL-6,IL-8, IL-10, IL-13, IL-17a, TRAIL, IFN-g, TNF-a, G-CSF, GM-CSF, VEGF, sTNFR-I, sTNFR-II, sRAGE, MCP-1, sCD40L) in sera of 19 patients treated with CyA (4mg/kg/day, p.o.,14days) after additional IVIG. Trough concentration of CyA in blood was kept between 80-200ng/mL. We examined serum samples before (day1), on day7, and at the end (day14) of CyA treatment. Assay was performed with Milliplex kit®. Fourteen patients (74%) responded to CyA and became afebrile within five days (Responders), whereas five (26%) patients were considered as non-responders. Serum transitional levels of IL-6, sIL-2R, G-CSF, VEGF, and sTNFR-II seemed to reflect disease severity. In non-responders, average levels of IL-6 (43.5 vs 13.8pg/ml, p<0.001) and sIL-2R (39.2 vs 10.7pg/ml, p<0.05) at day7 were significantly higher than those in responders. On days7 and14, levels of sIL-2R in 7 out of 14 responders were under the detection threshold, whereas those in non-responders were all in the detectable range.
(Conclusion) CyA treatment effectively reduced the persisting serum inflammatory cytokines in majority of the IVIG-refractory KD patients. Serum levels of IL-6 and sIL-2R may be good indicators of the efficacy of CyA treatment for refractory KD.
- © 2011 by American Heart Association, Inc.