THU0320 RISK FACTORS OF INTRAVENOUS IMMUNOGLOBULIN RESISTANCE AND CORONARY ARTERIAL LESIONS IN TURKISH CHILDREN WITH KAWASAKI DISEASE
Authors: Serkan Turkucar, Kaan Yildiz, Ceyhun Acari, Hatice Adiguzel Dundar, Mustafa Kir, Erbil Unsal
Journal: Annals of the Rheumatic Diseases (2019)

<h3>Background</h3> Kawasaki Disease (KD) is an acute, systemic, febrile vasculitis that occurs during infancy and is the most common cause of childhood coronary artery disease. The incidence of coronary artery lesions (CALs) has declined with the routine use of intravenous immunoglobulin (IVIG) treatment, but there is still considerable risk for resistance to IVIG treatment and development of CALs (1). <h3>Objectives</h3> Previously defined risk scoring systems have limited predictive capacity for IVIG resistance of KD in Turkish children. The present study was aimed to determine the risk factors in Turkish children with IVIG resistant KD and coronary artery involvement. <h3>Methods</h3> Clinical, laboratory and echocardiographic data were retrospectively analyzed in 94 Kawasaki patients. IVIG resistant and responsive groups were compared. <h3>Results</h3> Of the 94 patients included in the study, 55 (58.5%) were male and 39 (41.5%) were female and the ratio was 1.41. The median (25-75 percentage) age at the time of diagnosis was 35 (19-52) months. CALs were observed at echocardiographic evaluation in 31 patients (33%) and 17 patients (18.1%) were IVIG resistant. IVIG resistant group had a higher rate of CALs compared to the IVIG responsive group (p&lt;0.05). When two groups were compared prior to IVIG, neutrophil/lymphocyte ratio (NLR) and C-reactive protein (CRP) parameters were statistically higher in IVIG resistant group. NLR values still remained as statistically higher in the IVIG-resistant group after initial IVIG. Regarding IVIG resistance, duration of fever ≥ 9.5 days, CRP ≥ 88 mg/L and NLR ≥1.69 were the best cutoff values. The criteria for at least two of these three predictors were considered to be statistically significant risk factors for detecting IVIG resistance in KD before treatment (76.47% sensitivity, 71.05% specificity and 95% confidence intervals were 50.1-93.19% and 59.51-80.89%, respectively). Regarding risk for CALs, duration of fever ≥ 9.5 days before IVIG, (OR: 3.4) and Plt count after IVIG ≥ 670x10<sup>3</sup>/uL, (OR: 5.5), were the best cutoff values. <h3>Conclusion</h3> This study defined three criteria for IVIG resistance in KD prior to treatment: Duration of fever before IVIG ≥ 9.5 days, CRP ≥ 88 mg/L and NLR value ≥ 1.69. Presence of two of these three criteria were found as a significant risk factor for IVIG resistance. Following initial therapy with IVIG, if NLR value is ≥ 1.25, it also predicted ongoing inflammation and IVIG resistance, possibly a need for steroid therapy instead of second IVIG. Based on the clinical and laboratory features, we established a new risk-scoring system for predicting IVIG resistance in Turkish children with KD. This may be useful for choosing optimal treatment for KD before coronary artery involvement. <h3>Reference</h3> [1] Newburger JW, Takahashi M, Burns JC, Beiser AS, Chung KJ, Duffy CE, Glode MP, Mason WH, Reddy V, Sanders SP, et al. The treatment of Kawasaki syndrome with intravenous gamma globulin. N Engl J Med. 1986 Aug 7 ve 315(6):341-7. <h3>Disclosure of Interests</h3> Serkan Turkucar: None declared, Kaan Yildiz: None declared, Ceyhun Acari: None declared, Hatice Adiguzel Dundar: None declared, Mustafa Kir: None declared, Erbil Unsal Grant/research support from: Novartis, AbbVie, Roche, Koçak Pharma, Speakers bureau: Novartis, AbbVie, Roche, Koçak Pharma