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14-03-2019 | Kawasaki disease | News

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

medwireNews: Findings from the KAICA trial suggest that adding ciclosporin to intravenous immunoglobulin (IVIG) treatment may reduce the risk for coronary artery abnormalities among patients with Kawasaki disease.

However, the trial results “raised the possibility that patients treated with IVIG plus ciclosporin rather than IVIG alone are more susceptible to Kawasaki disease relapse after termination of the primary therapy,” say Akira Hata (Chiba University, Japan) and fellow researchers.

The study included 173 Japanese children and adolescents with Kawasaki disease who were predicted to be at elevated risk for nonresponse to IVIG based on a risk scoring system taking into account blood test results and patient characteristics. Participants were randomly assigned to receive ciclosporin at a dose of 5 mg/kg per day for 5 days in addition to IVIG 2 g/kg for 24 hours and aspirin 30 mg/kg per day, or to receive only IVIG and aspirin.

As reported in The Lancet, 14% of the 86 participants in the ciclosporin group experienced the primary endpoint of coronary artery abnormalities detected by echocardiography over 12 weeks of follow-up, compared with 31% of the 87 patients in the control group, a significant difference.


The goal in pediatric rheumatology is to have 0% of patients with coronary artery abnormalities.

Click here for the view of Editorial Board member Sangeeta Sule


Participants receiving ciclosporin also had significantly lower rates of nonresponse to primary therapy (17 vs 37%) than those who did not, but were more than twice as likely to experience relapse of Kawasaki disease, defined as return of fever without another likely source after an afebrile period of 48 hours or more following treatment initiation (27 vs 8%). The incidence of adverse events was comparable among the ciclosporin and control groups, at 9% and 7%, respectively.

Writing in an accompanying comment, Brian McCrindle (University of Toronto, Ontario, Canada) and Anne Rowley (Northwestern University Feinberg School of Medicine, Chicago, Illinois, USA) say that “[t]he net benefit to risk balance [of ciclosporin treatment] is not yet certain, with somewhat equivocal benefit for coronary artery outcomes possibly offset by an increased risk of relapse.”

In addition, the KAICA investigators found no significant difference in mean Z scores for each coronary artery segment among the groups, nor in the proportion of patients with coronary artery abnormalities with a Z score of 2.5 or more. They point out, however, that fewer patients in the combined treatment group appeared to have a coronary artery Z score of 3.0 or more.

Changes in Z score are “a more valid measure of coronary artery involvement” than the primary endpoint, note the commentators.

They add that regardless of the definition, the occurrence of coronary artery abnormalities decreased over time and was not significantly different between the groups by the 12-week follow-up, “suggesting that nearly all the involvement was transient.”

Nonetheless, McCrindle and Rowley believe that the results “provide important evidence to guide therapeutic advances, and the authors are to be congratulated for this important and carefully executed trial.”

The study authors conclude: “Further investigation is warranted to optimize the use of IVIG plus ciclosporin and determine which Kawasaki disease patients will most benefit from this combination as an initial therapy.”

By Claire Barnard

medwireNews is an independent medical news service provided by Springer Healthcare. © 2019 Springer Healthcare part of the Springer Nature group

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