Extracardial Vasculopathy After Kawasaki Disease: A Long‐Term Follow‐up Study

Abstract

BackgroundKawasaki disease (KD) is a pediatric vasculitis with coronary artery aneurysm (CAA) as a major complication. Controversy exists about cardiovascular risk later in life. The aim of our study was to evaluate whether KD patients are at increased risk, as assessed by carotid intima‐media thickness (cIMT).Methods and ResultsWe measured cIMT over 15 years by B‐mode ultrasonography in KD patients during follow‐up and in unaffected controls (mostly siblings). A multilevel, repeated‐measures, linear mixed‐effects model was used to evaluate the association between KD and cIMT. A total of 319 patients with 528 measurements were compared with 150 controls. In KD patients, the mean cIMT was increased compared with controls (0.375 mm [95% CI 0.372–0.378 mm] versus 0.363 mm [95% CI 0.358–0.368 mm]; P<0.001). Furthermore, mean cIMT of CAA‐negative patients was 0.373 mm (P<0.01 compared with controls), of patients with small–medium CAA was 0.374 mm (P<0.05 compared with controls), and of patients with giant CAA was 0.381 mm (P<0.01 compared with controls). Compared with controls, CAA‐negative participants started with an increased cIMT (+0.0193±0.0053 mm, P<0.001) but showed slower progression (−0.0014±0.0006 mm/year, P=0.012). Patients with giant CAA showed a trend toward increased cIMT progression (0.0013±0.0007 mm/year, P=0.058).ConclusionsWe observed a positive correlation between cIMT and KD severity of coronary arteritis at the acute stage. Although initially increased, the cIMT in CAA‐negative patients normalized at a later age. In contrast, patients with a history of KD complicated by giant CAA showed a trend toward persistently increased cIMT. These patients may need cardiovascular counseling and follow‐up beyond the heart.

Linkage and Association Analysis Identifies TRAF1 Influencing Common Carotid Intima–Media Thickness

“…40 Former KD patients might have an increased predisposition to atherosclerosis and endothelial damage. 40 Although still controversial 41 and possibly depending on the magnitude of the coronary artery aneurysm caused by KD, 42 some studies report increased CIMT in acute and healed KD. [42][43][44] The linkage analysis for CIMT identified a region on chromosome 4 with the LOD-1 support interval spanning 14 Mb encompassing 74 genes.…”

Recent updates on echocardiography and ultrasound for Kawasaki disease: beyond the coronary artery

“…Although the CIMT of patients without CCAs initially increases, the values normalize at a later age, suggesting vascular repair of a generalized vasculopathy. Patients with giant or medium-sized CAAs show a trend toward a continually increasing CIMT, suggesting a more marked impact on the arterial wall (29 (30). Although these studies have major flaw on the lack of the standardization of the CIMT using the z-score of CIMT (31), these highlight the importance of prospective tracking of this subset of KD patients with CAAs and an increased CIMT for the stratification of future CVD risk.…”

Kawasaki disease: what the paediatrician needs to know

“…Dietz et al seek to summarise recent advances in this state-of-the-art review [2]. Further, an increasing number of patients with Kawasaki disease now reach adulthood, and as a result, long-term prognosis has become an important discussion point for all specialists caring for these patients [1]. A succinct summary of data on long-term follow-up in patients with Kawasaki disease including their cardiovascular risk, issues surrounding quality-of-life, and its impact on social behaviour is provided and will be helpful for discussion with patients and families.…”