Abstract Body (Do not enter title and authors here): 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.