Cyclosporine Treatment in Patients with Kawasaki Disease and Coronary Artery Aneurysms or Treatment Resistance

OBJECTIVE: To describe the clinical course and outcome of 33 patients with Kawasaki disease (KD) treated with cyclosporine (CSA) for coronary artery abnormalities (CAA) or treatment resistance. STUDY DESIGN: Single-center, retrospective study of patients with KD treated from 2013 through 2023 for CAA or treatment resistance. Demographics, laboratory studies, medications, adverse events, and echocardiographic data were analyzed. RESULTS: Of the 33 KD patients treated with CSA, 25 patients received CSA for CAA and 8 for treatment resistance. Four patients received CSA intravenously initially, followed by oral therapy. Since 2014, all patients received CSA orally only. The target 2-hour postdose level (300-600 ng/ml) was best achieved by dividing 5 mg/kg/day every 12 hours. We developed a standardized treatment protocol based on our experiences with this patient population. The patients treated for CAA all showed improved coronary artery Z-scores at both the 2-week and 6-week follow-up. Two patients experienced significant adverse events. One patient had posterior reversible encephalopathy syndrome, while the other developed Epstein-Barr virus-associated lymphoproliferative disorder. Discontinuation of CSA led to complete recovery in both cases. CONCLUSIONS: CSA was generally well tolerated in patients with KD and CAA or treatment resistance. Our study contributes to the limited literature on CSA use in KD, providing dosing guidance and advocating for cautious monitoring.