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14-03-2019 | Article

Editorial board comment

Sangeeta Sule

Comment on: IVIG–ciclosporin combination may improve coronary artery outcomes in Kawasaki disease

The investigators in this study evaluated traditional therapy with intravenous immunoglobulin (IVIG) versus IVIG plus ciclosporin for treatment of Kawasaki disease and prevention of coronary artery abnormalities. This randomized, open-label, blinded study was an impressive undertaking, enrolling 175 participants.

For me, the interesting thing about this study is the fact that the researchers studied ciclosporin based on genetic studies showing possible upregulation of calcium-nuclear factor of activated T cells pathway. Using genetics to guide therapy is novel and, I hope, the wave of the future.

Kawasaki disease can cause coronary artery abnormalities in about 25% of untreated patients, and up to 20% of patients treated with traditional IVIG will develop coronary artery complications. The goal in pediatric rheumatology is to have 0% of patients with coronary artery abnormalities.

With this goal in mind, researchers have been exploring additional treatment modalities. Patient compliance is always something to consider when suggesting treatments, and an important take-away point for me from this study was that ciclosporin was given at a dose that was easy to monitor. The trough levels for ciclosporin were 60-200 ng/mL and the dose could be adjusted to maintain these levels.

The other important point from this study is that there is much we do not understand about Kawasaki disease. The investigators note that this study concentrated on the Japanese population and may not be generalizable to Europeans. Even with the reduction in coronary artery abnormalities noted with ciclosporin, almost 17% of participants in this group developed disease. Why? Further research is needed to explore the pathogenesis and predict treatment responses in this rare disease.