OBJECTIVE：To systematically evaluat e the efficacy and safety of different regimens in the treatment of refractory Kawasaki disease ，and to provide evidence-based reference for clinical treatment. METHODS ：Retrieved from PubMed ，Embase， Cochrane Library ， CNKI， VIP， Wanfang database ，randomized controlled trials （RCTs）and cohort studies about different therapeutic regimens in the treatment of refractory 84206032。E-mail：liuyingzryy@163.com Kawasaki disease were collected during the inception to March 2021. After selecting the literature and extracting the data ，the quality of RCT was evaluated by modified Jadad scale ，and the quality of cohort st udy was evaluated by NOS scale. Network Meta-analysis was performed by using Stata 16.0 software. RESULTS ：A total of 29 literatures were included ，involving 15 RCTs and 14 cohort studies. A total of 3 112 patients and 12 therapeutic regimens were involved ，including twice IVIG ，twice IVIG+hormone，twice IVIG+ulinastatin ，first IVIG ，first time IVIG+hormone ，first time IVIG+cyclosporine ，first time IVIG+ etanercept，hormone，hormone+ulinastatin，ulinastatin，infliximab and placebo. The results of network Meta-analysis showed that in terms of the incidence of coronary artery injury （CAL），twice IVIG+hormone was significant lower than hormone ，and first time IVIG +etanercept was significant lower than first time IVIG （P＜0.05）. The sorting results of network Meta-analysis showed that area under cumulative ranking curve of CAL incidence in ascending order was hormone ＜ulinastatin＜twice IVIG ＜first time IVIG＜first IVIG+hormone ＜twice IVIG+hormone ＜infliximab＜first time IVIG+cyclosporin ＜first time IVIG+etanercept. In terms of the incidence of ADR ，compared with twice IVIG+ hormone and hormone ，twice IVIG and first time IVIG+etanercept were decreased significantly ；infliximab was significantly lower than hormone （P＜0.05）. The sorting results of network Meta-analysis showed that area under cumulative ranking curve of ADR incidence in ascending order was hormone ＜twice IVIG+hormone ＜first time IVIG+hormone ＜first time IVIG+cyclosporin ＜first time IVIG ＜twice IVIG ＜first time IVIG+etanercept ＜infliximab. In terms of the serum level of CRP ，compared with twice IVIG ，twice IVIG+hormone ，twice IVIG+ulinastatin and hormone were decreased significantly；twice IVIG+hormone was significantly lower than first time IVIG ；twice IVIG+ulinastatin were all significantly lower than twice IVIG+hormone ，hormone，hormone+ulinastatin，first time IVIG ，first time IVIG+hormone and ulinastatin （P＜0.05）. The sorting results of network Meta-analysis showed that area under cumulative ranking curve of serum CRP level in ascending order was first time IVIG ＜first time IVIG+hormone ＜twice IVIG ＜hormone+ulinastatin＜ulinastatin＜infliximab＜hormone＜twice IVIG+hormone＜twice IVIG+ulinastatin. In terms of improving persistent fever duration ，there was no statistical difference between pairwise treatment measures （P＞0.05）. The sorting results of network Meta-analysis showed area under cumulative ranking curve of persistent fever time in ascending order was first time IVIG ＜placebo＜first time IVIG+cyclosporine ＜hormone＜twice IVIG+ hormone＜twice IVIG ＜ulinastatin＜infamliximab. CONCLUSIONS ：The first time IVIG+etanercept has the best effect in reducing the incidence of CAL. Infliximab possesses a relatively low incidence of ADR and the best antipyretic effect. Twice IVIG + ulinastatin has the best anti-inflammatory effect.