Treatment and Coronary Artery Aneurysm Formation in Kawasaki Disease: A Per-Day Risk Analysis

Diana Van Stijn,Justin M Korbee,Stejara A Netea,Vera C De Winter,Koos A.H Zwinderman,Irene M Kuipers,Taco W Kuijpers

doi:10.1016/j.jpeds.2021.12.054

Diana Van Stijn, Justin M Korbee + Show 5 more

Open Access

https://doi.org/10.1016/j.jpeds.2021.12.054

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Abstract

To assess whether 'treatment day' is a significant predicting factor in Kawasaki disease and imposes a risk for coronary artery aneurysms (CAAs) in a per-day analysis. CAA formation can be reduced from 25% to 10% when treated with intravenous immunoglobulin (IVIG). Patient data from (n=1016) a single center were collected for an observational cohort study. After exclusions, we retrospectively analyzed 776 patients in total. A multivariate analysis was performed with treatment day as a continuous variable (n=691). Patients were categorized as no enlargement, small CAA, medium CAA, and giant CAA. Late treatment per-day was a significant predicting factor for the development of larger CAAs. ORs for medium and giant CAAs per delayed day were 1.1 (95% CI 1.1-1.2) P<.05 and 1.2 (95% CI 1.1-1.2) P<.05, respectively. We showed that every day of delay in treatment of patients with Kawasaki disease inherently carries a risk of medium and giant aneurysm formation. There was no cut-off point for treatment day that could mark a safe zone.

Highlights

Post mortem studies have identified pathologic vascular processes causing arterial wall destruction potentially leading to aneurysm formation, such as necrotizing arteritis, and a subacute/chronic vasculitis, recognized by infiltration of lymphocytes, plasma cells, eosinophils, and macrophages.[1]
Occlusion of a coronary artery can occur due to thrombosis caused by abnormal blood flow in the coronary artery aneurysms (CAAs) or arterial stenosis caused by luminal myofibroblastic proliferation.[1,2,3,4]
CAAs can be expressed in z scores, which are adjusted for the patients’ body-surface-area

Summary

Methods

Record files from patients meeting the diagnostic criteria for Kawasaki disease[31] that were referred to the national expertise center for Kawasaki disease in the Netherlands (at diagnosis or during follow-up) until April 1, 2020, were anonymously used for this study. From the 1Department of Pediatric Immunology, Rheumatology, and Infectious Diseases, Emma Children’s Hospital, 2Department of Public Health and Clinical Epidemiology, and 3Department of Pediatric Cardiology, Emma Children’s Hospital, Amsterdam University Medical Center, University of Amsterdam, Amsterdam, The Netherlands. Supported by the foundation “Kind en Handicap” and an anonymous donor through the Amsterdam Medical Centre foundation. The authors declare no conflicts of interest.

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Discussion

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