The Predictive Value Of Scoring Systems On Intravenous Immunoglobulins Resistant Kawasaki Disease And System Evaluation On 269 Cases Of Kawasaki Disease Treated By Plasma Exchange

ObjectiveThe aim of this study was to investigate the incidence rateand the risk factors of intravenous immunoglobulins(IVIG)resistantin Kawasaki disease,and evaluate the predictive efficiency of the Kobayashi and Egami scoring systems.MethodsFrom Jan 2017 to Dec 2017,the clinical and laboratory features of 247 KD patients in ourhospital were retrospectively analyzed.According to the IVIG-resistant KD diagnostic standards defined in the Diagnosis,Treatment and Long-Term Management of KD issued by AHA in 2017,all the cases can be divided into IVIG-resistant group and IVIG sensitive group.The study analyzed and compared the results including clinical features,laboratory indicators,coronary artery dilation in two groups.Kobayashi,Egami scoring systems were applied to all patients to checkoutthe predictive value of IVIG-resistant KD.ResultsThere were 247 cases of KD children patients receiving the first dose of IVIG therapy.22 cases belong to the IVIG-resistant group(8.91%),225 cases belong to the IVIG sensitive group(91.09%).There were 9 cases of male patients in the IVIG-resistant group(40.91%)and 142 cases of male patients in the IVIG sensitive group(63.11%).There were statistical differences(?2=4.158,P=0.041)between two groups.There was no obvious difference in the incidence rate of clinical features,the total number of clinical features and abnormalities of the coronary artery between the two groups(P>0.05).The single factor analysis revealed that there was statistical difference in the low levels of PLT,Alb and blood sodium(P<0.05).After the treatment of IVIG,the decreases of WBC,NE,CRP in the IVIG-resistant were not obvious and hypoproteinemia still existed.There was statistical difference between two groups(p<0.05).Logistic regression analysis finded that after treatment,the high levels of NE and CPR were the independent risk factors in IVIG-resistant KD.ROC curve was used to evaluate the efficacy oftwopredict scores of IVIG-resistant KD.The AUC of Kobayashi scores was 0.502,it revealed thefailure of prediction(P = 0.973);The AUC of Egami scores was 0.673 and it can partly predict IVIG-resistant KD(P = 0.008).When the cutoff value is 3,the sensitivity was 22.7% and the specificity was 92.8%.Conclusion(1)After the initial IVIG treatment,the incidence rate of unresponsive KD was 8.91%.One year's data of single center shown: The incidence rates of clinical features,the total number of clinical features and the coronary artery disease were almost the same as the sensitive group.(2)There were higher risks of hypoproteinemia and hyponatremia in IVIG-resistant KD children patients.After the IVIG treatment,WBC,NE,CRP were not significantly decreaseand hypoproteinemia still exists.That might reveal the possibility of IVIG-resistance.(3)The high levels of NE and CRP after IVIG were the independent risk factors in IVIG-resistant KD.(4)Kobayashi scoring system's efficacy of predicting IVIG-resistance was low;Egami scoring systemwas valuable to some patients,but it's application is limited due to it's low sensitivity.BackgroundKawasaki disease is a self limited disease,which is mainly manifested by systemic immune vasculitis.The most serious complication is coronary artery injury.At present,the pathogenesis of KD is not completely clear,but it is sure that there are inflammatory cells such as B cells and T cells,as well as a large number of inflammatory factors such as IL-6,IL-8,IL-10,IL-17,TNF-alpha and so on.These factors cause the metabolic disorder of the extra-vascular matrix and lead to the destruction of the basement membrane and internal elastic membrane,coronary artery dilatation and coronary artery aneurysm.Studies had shown that the children with immunoglobulin refractory Kawasaki disease(IVIG-resistant KD)had higher level of inflammatory factors and higher incidence of coronary artery injury was higher,than IVIG sensitive patients.At present,a lot of studies have been done on the treatment of IVIG refractory KD,but there are still some KD children who have no response to the first dose IVIG treatment and had coronary artery injury(CALs).Plasma exchange is the only non-drug therapy in the current treatment of KD.The mechanism of plasma exchange is used to remove the macromolecular substances and inflammatory cytokines in the blood circulation and prevent cardiovascular sequelae.For children with IVIG-resistant KD,PE is one of the most important methods for the IVIG-resistant KD for the rapid removal of abnormal inflammatory cytokines and super antigen and other humeral factors.Researchers shoulduse plasma exchange technology rationally in KD patients and reduce complication.ObjectiveSystematic evaluation was made on the indications,treatment methods,efficacy and complication of PE in KD patients.MethodIn this study,269 cases of KD with the treatment of PE from 2004 to 2015 were analyzed the studies about plasma exchange in KD patients were scanned in the database.The database included Pubmed,Embase,Cochrane Library,Chinese biomedical literature database(CBM),knowledge network database,Wanfang database and Chongqing VIP number.The indications,contraindications,methods,effects and complication of plasma exchange in KD patients were summarized to guide the use of plasma exchange therapy.Results(1)Indication and contraindication: It was suitable for the useless of second dose IVIG,long-term hormone therapy,and ineffective KD for infliximab;it was also applicable to children of small age,light weight,and KD children with cardiac insufficiency or unsuitable drugs.PE did not have absolute contraindications,and have few relatively contraindications.(2)Efficacy: Before PE,KD patients had persistent fever and high expression of inflammatory factors or had existed CALs.After PE treatment,fever were subsided and inflammatory factors decreased significantly.Some patients with CALs was recovered in the long-term follow-up: 73 of 269 KD patients were not followed up,and some of the other patients had CALs in the acute period,but only 6 cases of CALs(1 coronary aneurysm and 5 giant coronary aneurysms)were left in the later period.The effective rate of plasma exchange therapy for IVIG reactive Kawasaki disease(ES=0.97;95%CI: 0.92-1.00)was statistically significant.(3)Complication: Only 4 of 269 KD patients with PE had complication,one casetreated with mechanical ventilation were infected by respiratory syncytial virus and had laryngitis,2 cases hadhypotension and 1 case had pneumothorax,all of the 4 patients recovered after symptomatic treatment without serious adverse events.ConclusionIn most cases,PE therapy can improve the acute inflammatory reaction of severe Kawasaki disease.(1)PE treatment was suitable for the following children: IVIG-resistant KD children with severe inflammatory response and high risk factors for coronary artery injury,especially KD with poor therapeutic effects on multiple or multiple drugs,or children with small age,weight loss or with cardiac insufficiency or unsuitable drugs.(2)PE in the treatment of IVIG-resistant KD was efficacy and safe.Combined with PE therapy on the basis of conventional drugs may be one of the safe and effective treatment schemes for refractory KD.(3)PE in pediatric ICU with well equipped and developed medical institutions can prevent the occurrence of adverse events.