Abstract
Kawasaki disease (KD) is a febrile disease of childhood characterized by systemic vasculitis that can lead to coronary artery lesions (CAL). This was a prospective cohort study to determine the levels of the pentraxin 3 (PTX3), soluble CD24-Subtype (Presepsin) and N-terminal pro-brain natriuretic peptide (NT-pro BNP) in consecutive KD patients. From January 2013 to March 2015, all patients with KD admitted to Aichi Medical University Hospital who provided consent had their plasma saved before IVIG administration. In total, 97 cases were registered. 22 cases of incomplete KD were excluded from the outcome analysis. The total 75 cases were used for statistical analyses. A PTX3 threshold of >7.92 ng/ml provided a specificity of 88.5 %, a sensitivity of 94.4 %, and a likelihood ratio as high as 15.92 for the diagnosis of KD compared with febrile non-KD controls. Although an echocardiographic diagnosis of CAL in the early course of the disease was confirmed in 24 cases, it was not in the remaining 51 cases. Neither NT-proBNP nor Presepsin had statistical significance for the prediction of the echocardiographic CAL diagnosis. Only PTX3 was significantly predictive of the echocardiographic CAL diagnosis (p=0.01). The PTX3 level was significantly higher in the intravenous immunoglobulin (IVIG) non-responders (45.9±7.45) than in the IVIG responders (17.0 ± 1.46 ng/ml) (p< 0.001). The PTX3 level also correlated with the number of IVIG treatment courses needed to resolve fever (R² =0.64). Persistent CAL (pCAL) formation was observed in three cases; one of aneurysm only and two aneurysms with dilatations. The patients with pCAL had significantly higher PTX3 levels (85 ± 8.4 ng/ml) than patients without pCAL (22 ± 2.2 ng/ml) (p< 0.0001). In terms of pCAL prediction, the area under the curve (AUC) of receiver operating characteristic ROC curve of PTX3 was 0.99, and it was significantly greater than that of Presepsin (0.67) or NT-proBNP (0.75). PTX3 is a soluble pattern recognition molecule that acts as a main component of the innate immune system. These data suggest that PTX3 can be utilized as a definitive biomarker for the prediction of IVIG resistance and subsequent CAL formation in patients with KD.
Highlights
Kawasaki disease (KD) is an acute inflammatory disorder of unknown origin associated with medium-sized vessel vasculitis [1]
All of the KD patients were treated with oral aspirin (30 mg/ kg per day) and intravenous immunoglobulin (IVIG) (2 g/kg per day). 115 cases were registered in total. 18 cases that presented with fever, but with symptoms that were not compatible with KD, were excluded. 22 cases of incomplete KD were excluded from the outcome analysis. 41 boys and 34 girls fulfilling the standard diagnostic criteria for KD were analyzed in the study
Plasma exchange was performed in two patients after three doses of IVIG
Summary
Kawasaki disease (KD) is an acute inflammatory disorder of unknown origin associated with medium-sized vessel vasculitis [1]. Inflammation of medium-sized arteries throughout the body, of the coronary arteries, can occur during the acute phase of KD [2], and coronary artery aneurysms develop in the subacute phase [3]. Coronary artery lesions (CAL), including dilations and aneurysms, respectively manifesting in the acute and transient form or persistent chronic form (designated as persistent CAL; pCAL), constitute the most serious complication. PCALs are the most common cause of acquired heart disease in children in developed countries [4, 5]. Coronary artery aneurysms develop in 15–25% of untreated children and may lead to myocardial infarction, sudden death, or ischemic heart disease [6]. Mortality may approach 1%, usually within six weeks of onset [7]
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