Clinical Outcomes of Cyclosporine Treatment in Kawasaki Disease

The current retrospective study aimed to outline the clinical progression and outcomes of 33 patients with Kawasaki disease (KD) who were treated with cyclosporine (CSA) due to coronary artery abnormalities (CAA) or resistance to treatment.The researchers examinedKD patients treated between 2013 and 2023 for CAA or treatment resistance. The analysis included demographics, lab results, medications, adverse events, and echocardiographic findings.

The results revealed:

Among the 33 KD patients treated with CSA, 25 were treated for CAA and 8 for treatment resistance.
Initially, four patients received CSA intravenously, transitioning to oral treatment later; since 2014, all patients have been treated orally.
The optimal 2-hour post-dose level (300-600 ng/ml) was best achieved with a regimen of 5 mg/kg/day divided every 12 hours.
A standardized treatment protocol was developed based on our clinical experience.
Patients with CAA demonstrated improved coronary artery Z-scores at both the 2-week and 6-week follow-ups.
Two significant adverse events were recorded: one patient developed posterior reversible encephalopathy syndrome, and another had Epstein-Barr virus-associated lymphoproliferative disorder; both fully recovered after stopping CSA.

Thus, the study concluded that CSA is well tolerated in patients with KD experiencing CAA or treatment resistance. This study adds to the limited existing literature on the use of CSA in KD, offering dosing recommendations and emphasizing the need for careful monitoring.

Source: Bellicini I, Bainto E, Shimizu C, Burns JC, Tremoulet AH. Cyclosporine Treatment in Patients with Kawasaki Disease and Coronary Artery Aneurysms or Treatment Resistance. J Pediatr. 2025 Apr;279:114479. doi: 10.1016/j.jpeds.2025.114479. Epub 2025 Jan 24. PMID: 39863079.

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