Abstract 4143704: Systemic Arterial Aneurysms in Kawasaki Disease: An Important Evidence Gap

Abstract

Introduction: Non-coronary artery systemic arterial aneurysms (SAA) are a rare and under-reported sequelae of Kawasaki disease (KD), for which data and guidance are limited. Methods: A survey was sent to members of the International KD Registry (IKDR) regarding their experiences and practices with SAA in KD patients. For comparison, a systematic review was conducted following PRISMA methodology; after evaluation, a total of 21 studies with 75 patients total were included. Results were compared qualitatively. Results: Surveys were completed by 48 (56%) of 86 IKDR investigators; 35 (73%) respondents had >10 years of experience caring for KD patients. Experience with SAA was limited, with 33% not having cared for a patient with SAA. Reported screening practices included 81% screening only patients with coronary artery (CA) involvement, 8% reporting screening all patients with KD, and with 11% using other criteria. The degree of CA involvement influenced screening, with 56% screening only those with giant CA aneurysms, 20% also small/medium aneurysms, 6% included dilation and 18% not using any specific CA criteria. Additional factors reported included multiple or rapidly expanding CA aneurysms, clinical features such as prolonged/persistent fever, progressing/persistent elevation of inflammatory markers, and resistance to standard treatment. These screening practices were somewhat concordant with the characteristics of patients with SAA reported in the literature ( FIGURE ). Initial preferred assessment method was CT angiography for 48%, ultrasound 28% and MRI for 24% of respondents. In contrast, SAA reported in the review were most commonly assessed with conventional coronary artery angiography. From the review, SAA were associated with a high rate of regression (62-93%). Longer-term complications reported included SAA thrombosis, calcification, stenosis, occlusion and collateral formation. Clinical and management factors associated with SAA outcomes were not defined. Conclusion: While the development of SAA is a known but rare complication of acute KD, there remains a gap in evidence as to which patients are at risk, best practices for screening and management, and outcomes. Protocol driven cohort studies are needed.