Background:
Statins have been considered as therapy for children with coronary artery aneurysms (CAA) after Kawasaki disease (KD), due to potential beneficial pleiotropic effects which might influence chronic vascular processes and inflammation.
Methods:
The North American Kawasaki Disease Registry was queried to identify patients who have received statins in the first 6 months following the convalescent phase of KD. Each identified patient was matched by age, gender and CAA z score to 3 patients who were statin-naïve (controls). Linear regression models adjusted for repeated measures and maximum coronary involvement were used to determine an association of statin use with longitudinal changes in coronary artery diameter z-score. Kaplan-Meier analysis was used to compare freedom from angiographically-confirmed stenosis or interventions.
Results:
Of 29 patients with KD and CAA (maximum coronary artery z-score >10) who received statins at any time (of n=621, 5%), 10 (9 males) patients were started within 6 months of the acute KD episode. The mean age at KD was 6.3±3.4 years (5.4±3.5 for controls, p=0.57). Mean maximum CAA z-score was 36±14 (vs. 29±16, p=0.20); 90% of statin patients and 87% of matched controls had CAAs in 3 or more branches. Linear regression analysis of 442 serial echocardiograms showed that maximum CAA z-score decreased by -1.5 (95%CI: -2.7; -0.4) SD/year (p=0.008) for control patients compared to -2.9 (95%CI: -4.4; -1.4) SD/year (p<0.001) for statin treated patients. The difference between the rate of change of CAA z-score for statin vs. control patients did not reach statistical significance (controls vs. statins: +1.4 SD/year, 95%CI: -0.6; +3.4, p=0.18). n=7 patients (3 on statin, 4 controls) developed stenosis or had revascularization, with no significant difference between groups (HR for statin group: 2.2 (0.4-11.4), p=0.41).
Conclusions:
This underpowered pilot study suggests that equipoise likely exists with regards to statin therapy in children with KD and CAA, and that a formal registry-nested trial might be considered.
Torticollis as Presentation for Atypical Kawasaki Disease Complicated by Giant Coronary Artery Aneurysms
