Ryohei Matsuoka1, Kenji Furuno2, Etsuro Nanishi3, Sagano Onoyama4, Hazumu Nagata3, Kenichiro Yamamura3, Yuichiro Sugitani5, Ayako Kuraoka5, Yumi Mizuno4, Koichi Sagawa5, Satoshi Honjo6, Toshiro Hara4, Shouichi Ohga3. 1. Department of General Pediatrics and Interdisciplinary Medicine, Fukuoka Children's Hospital, Fukuoka, Japan; Department of Pediatrics, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan. 2. Department of General Pediatrics and Interdisciplinary Medicine, Fukuoka Children's Hospital, Fukuoka, Japan; Kawasaki Disease Center, Fukuoka Children's Hospital, Fukuoka, Japan. Electronic address: furuno.k@fcho.jp. 3. Department of Pediatrics, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan. 4. Kawasaki Disease Center, Fukuoka Children's Hospital, Fukuoka, Japan. 5. Department of Pediatric Cardiology, Fukuoka Children's Hospital, Fukuoka, Japan. 6. Department of Pediatrics, National Hospital Organization Fukuoka National Hospital, Fukuoka, Japan.
Abstract
OBJECTIVE: To clarify the frequency and characteristics of discrepant outcomes of intravenous immunoglobulin (IVIG) between fever and coronary artery aneurysms (CAAs) in patients with Kawasaki disease. STUDY DESIGN: This study included 325 patients who responded to oral aspirin and IVIG alone. The main outcome was CAA 4 weeks after disease onset. CAA was defined as ≥2.5 of maximum z score (Zmax) representing the highest value of 4 coronary artery branches. Immunoglobulin dosage and sequential changes in Zmax were reviewed to investigate the effects on fever and timing of CAA development. Logistic regression analyses with receiver operating characteristic curves using clinical and laboratory variables including the initial Zmax were performed to identify predictors of CAA at 4 weeks. RESULTS: CAAs were either persistent or appeared de novo 4 weeks after diagnosis in 13 of 325 patients who responded to a single or repeated IVIG. Four single-dose IVIG-responders developed CAA although they had pretreatment Zmax of <2.0. The 2 single-dose IVIG responders with the greatest pretreatment Zmax (>4.5) developed persistent CAA. Receiver operating characteristic analysis demonstrated Zmax of 2.57 as the cut-off for predicting CAA. Multivariable analyses identified >2.5 Zmax (OR 9.08, 95% CI 1.26-65.3, P = .028, 50% sensitivity, 91% specificity) as the sole risk factor for CAA at 4 weeks in single-dose IVIG responders. CONCLUSIONS: Delayed development and persistence of CAA in single-dose IVIG responders indicate that some factors other than those responsible for systemic inflammation may contribute to vasculitis in CAA. Baseline Zmax 2.5 aids in predicting CAAs.
OBJECTIVE: To clarify the frequency and characteristics of discrepant outcomes of intravenous immunoglobulin (IVIG) between fever and coronary artery aneurysms (CAAs) in patients with Kawasaki disease. STUDY DESIGN: This study included 325 patients who responded to oral aspirin and IVIG alone. The main outcome was CAA 4 weeks after disease onset. CAA was defined as ≥2.5 of maximum z score (Zmax) representing the highest value of 4 coronary artery branches. Immunoglobulin dosage and sequential changes in Zmax were reviewed to investigate the effects on fever and timing of CAA development. Logistic regression analyses with receiver operating characteristic curves using clinical and laboratory variables including the initial Zmax were performed to identify predictors of CAA at 4 weeks. RESULTS: CAAs were either persistent or appeared de novo 4 weeks after diagnosis in 13 of 325 patients who responded to a single or repeated IVIG. Four single-dose IVIG-responders developed CAA although they had pretreatment Zmax of <2.0. The 2 single-dose IVIG responders with the greatest pretreatment Zmax (>4.5) developed persistent CAA. Receiver operating characteristic analysis demonstrated Zmax of 2.57 as the cut-off for predicting CAA. Multivariable analyses identified >2.5 Zmax (OR 9.08, 95% CI 1.26-65.3, P = .028, 50% sensitivity, 91% specificity) as the sole risk factor for CAA at 4 weeks in single-dose IVIG responders. CONCLUSIONS: Delayed development and persistence of CAA in single-dose IVIG responders indicate that some factors other than those responsible for systemic inflammation may contribute to vasculitis in CAA. Baseline Zmax 2.5 aids in predicting CAAs.
Authors: Marcello Chiocchi; Carlo Di Donna; Alfredo Intorcia; Luca Pugliese; Vincenzo De Stasio; Federica Di Tosto; Luigi Spiritigliozzi; Francesca D'Errico; Leonardo Benelli; Monia Pasqualetto; Cecilia Cerimele; Matteo Cesareni; Francesco Grimaldi; Francesco Paolo Sbordone; Alessandra Luciano; Mario Laudazi; Carlotta Rellini; Alessia Romeo; Gianluca Vanni; Daniele Morosetti; Marco Di Luozzo; Roberto Floris; Francesco Romeo; Francesco Giuseppe Garaci Journal: Radiol Case Rep Date: 2021-05-01