Giant aneurysms: A gender-specific complication of Kawasaki disease?

Abstract

BackgroundKawasaki disease (KD) is a pediatric vasculitis of unknown origin. Its main complication is the development of coronary artery aneurysms (CAA) with giant CAA at the end of the spectrum. MethodsIn this cohort study, we evaluated the association between patient characteristics and the development of giant CAA based on z-scores. Multivariable, multinomial logistic regression analysis was used to identify variables associated with giant CAA. ResultsA total of 301 KD patients, comprising 216 patients without enlargement, 45 with small-sized, 19 with medium-sized, and 21 with giant CAA with all echocardiographies at our center were retrospectively included. Remarkably, 95% of patients with giant CAA were boys. In addition to ‘no/late intravenous immunoglobulin (IVIG) treatment’, ‘male gender’ (OR 16.23, 95% CI 1.88–140.13), ‘age<1 year’ (OR 7.49, 95% CI 2.29–24.46), and ‘IVIG re-treatment (9.79, 95% CI 2.79–34.37)’ were significantly associated with an increased risk of giant CAA, with patients without enlargement as reference. Compared to patients with medium-sized CAA, ‘IVIG re-treatment’ was significantly associated with giant CAA. The majority of giant CAA continued to increase in size during the first 40 days. ConclusionsWe identified risk factors associated with an increased risk of giant CAA. The difference in variables between the giant CAA group and the other CAA subgroups suggests a separation between patients with the treatment-resistant giant CAA and the other IVIG-responsive patients, in which gender may be factored as a most relevant genetic trait. The increase in size during the first 2 months indicates the need for repeated echocardiography.