Calcineurin Inhibitor Treatment of Intravenous Immunoglobulin–Resistant Kawasaki Disease

作者: Adriana H. Tremoulet , Paige Pancoast , Alessandra Franco , Matthew Bujold , Chisato Shimizu

DOI: 10.1016/J.JPEDS.2012.02.048

关键词:

摘要: Objective To describe the clinical course and outcome of 10 patients with Kawasaki disease (KD) treated a calcineurin inhibitor after failing to respond multiple therapies. Study design Demographic data were prospectively collected using standardized case report forms. T-cell phenotypes determined by flow cytometry, KD risk alleles in ITPKC (rs28493229), CASP3 (rs72689236), FCGR2A (rs1801274) genotyped. Results Intravenous followed oral therapy cyclosporine (CSA) or tacrolimus was well tolerated resulted defervescence resolution inflammation all patients. There no serious adverse events, treatment protocol developed based on our experiences this patient population. Analysis phenotype cytometry 2 subjects showed decrease circulating activated CD8 + CD4 T effector memory cells CSA. However, suppression regulatory T-cells not seen, suggesting targeting specific, proinflammatory compartments Conclusion Treatment refractory appears be safe effective approach that achieves rapid control associated improvement.

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