Kawasaki disease (KD) is an acute self-limiting vasculitis, and it is the most common cause of acquired heart disease in children under 5 years old. One of the improvement goals in pediatric quality control work for the year 2023, as announced by the National Health Commission, is to reduce the incidence of cardiac events and KD-related mortality in children with KD. In order to standardize the diagnosis, treatment, and long-term management practices of KD in China, and effectively prevent and reduce the incidence of coronary artery lesions and long-term adverse effects, the guideline working group followed the principles and methods outlined by the World Health Organization and referenced existing evidence and experiences to develop the \"Evidence-based guidelines for the diagnosis and treatment of Kawasaki disease in children in China (2023)\". The guidelines address the clinical questions regarding the classification and definition of KD, diagnosis of different types of KD, treatment during the acute phase of KD, application of echocardiography in identifying complications of KD, and management of KD combined with macrophage activation syndrome. Based on the best evidence and expert consensus, 20 recommendations were formulated, aiming to provide guidance and decision-making basis for healthcare professionals in the diagnosis and treatment of KD in children.
川崎病（Kawasaki disease, KD）是一种急性自限性血管炎性疾病，是引起5岁以下儿童后天获得性心脏病的最常见原因。国家卫生健康委员会发布的2023年儿科质控工作改进目标之一是降低KD患儿心脏事件的发生率及KD相关病死率。为规范我国KD的诊断、治疗和长期管理实践，有效预防、减少冠状动脉病变的发生及远期不良影响，指南工作组遵循世界卫生组织指南制订的原则和方法，参照现有国内外证据和经验形成了《中国儿童川崎病诊疗循证指南（2023年）》。该指南针对KD的分类及定义、不同类型KD的诊断、KD急性期治疗、超声心动图在KD并发症识别中的应用和KD合并巨噬细胞活化综合征的处理等临床问题，基于最佳证据和专家共识形成了20条推荐意见，以期为广大医护人员在儿童KD的诊断与治疗方面提供指导与决策依据。.

Kawasaki disease (KD) is a medium-sized vessel vasculitis of unknown origin that predominantly affects infants and young children. As KD causes cardiac complications such as coronary artery lesions, it is known as a disease that causes sudden death in children with acquired cardiac disease.
The clinical trials of prednisolone, infliximab and cyclosporin A have led to the insurance coverage of these drugs in the treatment of KD, in addition to intravenous immunoglobulin therapy, which was already indicated for the treatment of KD. Despite not being a drug, plasma exchange therapy as a procedure was also approved for insurance coverage in Japan. Furthermore, new guidelines for KD treatment were published by the American Heart Association in 2017 and the Single Hub and Access Point for Paediatric Rheumatology in Europe in 2019. In light of these circumstances, the Japanese Society of Pediatric Cardiology and Cardiac Surgery guidelines were revised.
Here, we provide an overview of the revised guidelines and mention the position and actual practice of plasma exchange therapy as one of the ultimate treatment modalities.

Kawasaki disease (KD) is one of the common acquired heart diseases in children aged <5 years and is an acute systemic vasculitis. After nearly 60 years of research, intravenous immunoglobulin combined with oral aspirin has become the first-line treatment for the prevention of coronary artery lesion in acute KD; however, there are still controversies over the role and optimal dose of aspirin. The consensus was formulated based on the latest research findings of KD treatment in China and overseas and comprehensive discussion of pediatric experts in China and put forward recommendations on the dose, usage, and course of aspirin treatment in the first-line treatment of KD.
川崎病（Kawasaki disease，KD）是5岁以下儿童常见后天获得性心脏病之一，是一种急性全身性血管炎。经过近60年的研究，静脉注射免疫球蛋白（intravenous immunoglobulin，IVIG）联合阿司匹林（aspirin，Asp）口服成为急性期KD预防冠状动脉病变（coronary artery lesion，CAL）的一线治疗。然而，Asp的作用和最佳剂量仍然存在争议。该共识基于KD治疗的国内外最新的研究成果，并经过国内儿科专家充分讨论而制定，对Asp在KD中一线治疗的使用剂量、用法、疗程等提出了推荐意见。.

Key aspects of the medical management of Kawasaki disease (KD) are not yet supported by a high evidence level, thus making room for individual recommendations. We performed a structured comparison of existing international KD guidelines to analyze potential differences in the implementation of evidence-based KD recommendations regarding diagnosis and therapy. To identify country-specific guidelines, we took a multilateral approach including a comprehensive PubMed literature, online research, and directly contacting national pediatric associations. We then ran a structured guidelines\' analysis and evaluated the diagnostic and therapeutic differences in the context of evidence-based medicine. In this structured guideline analysis, we identified nine national and one European guidelines. According to them all, the diagnosis of KD still relies on its clinical presentation with no reliable biomarker recommended. First-line treatment consistently involves only intravenous immunoglobulin (IVIG) therapy. Recommendations in terms of acetylsalicylic acid, corticosteroids, and additional therapeutic options vary considerably.
CONCLUSIONS: According to all guidelines, KD is diagnosed clinically with some variance in defining incomplete KD and being a non-responder to treatment. First-line treatment consistently includes IVIG. Recommendations for additional therapeutic strategies are more heterogeneous.
BACKGROUND: • The diagnosis of KD relies on the clinical presentation, entailing challenges in timely diagnosis. • Other treatment options then IVIG are not supported by a high evidence level, making room for individual recommendations.
BACKGROUND: • Definition of incomplete KD and being non-responsive to an initial treatment vary to some extent between the national guidelines. • Only IVIG is consistently proposed throughout all guidelines, further therapeutic recommendations vary between the national recommendations.

Kawasaki disease (KD) is one of the common acquired heart diseases in under-5-year-old children and is an acute self-limiting vasculitis. After nearly 60 years of research, intravenous immunoglobulin combined with oral aspirin has become the first-line treatment for preventing coronary artery aneurysm in the acute stage of KD. However, glucocorticoid (GC), infliximab, and other immunosuppressants are options for the treatment of KD patients with a high risk of coronary artery aneurysm, no response to intravenous immunoglobulin and a confirmed diagnosis of coronary artery aneurysm. At present, there are still controversies over the use of GC in the treatment of KD. With reference to the latest research findings of KD treatment in China and overseas, this consensus invited domestic pediatric experts to fully discuss and put forward recommendations on the indications, dosage, and usage of GC in the first-line and second-line treatment of KD.
川崎病（Kawasaki disease，KD）是5岁以下儿童常见后天获得性心脏病之一，是一种急性自限性血管炎。经过近60年的研究，静脉注射免疫球蛋白联合阿司匹林口服成为急性期KD预防冠状动脉瘤的一线治疗。但对于发生冠状动脉瘤高风险、静脉注射免疫球蛋白无反应、确诊冠状动脉瘤等KD患者，糖皮质激素（glucocorticoid，GC）、英夫利昔单抗及其他免疫抑制剂是可以选择的治疗药物。目前GC在KD治疗中的应用存在争议。该共识结合KD治疗的国内外最新的研究成果，邀请国内儿科专家充分讨论，对GC在KD中一线及二线治疗的适应证、剂量、用法等提出了推荐意见。.

To provide evidence-based recommendations and expert guidance for the management of Kawasaki disease (KD), focusing on clinical scenarios more commonly addressed by rheumatologists.
Sixteen clinical questions regarding diagnostic testing, treatment, and management of KD were developed in the Patient/Population, Intervention, Comparison, and Outcomes (PICO) question format. Systematic literature reviews were conducted for each PICO question. We used the Grading of Recommendations, Assessment, Development and Evaluation method to assess the quality of evidence and formulate recommendations. Each recommendation required consensus from at least 70% of the Voting Panel.
We present 1 good practice statement, 11 recommendations, and 1 ungraded position statement to guide the management of KD and clinical scenarios of suspected KD. These recommendations for KD are focused on situations in which input from rheumatologists may be requested by other managing specialists, such as in cases of treatment-refractory, severe, or complicated KD. The good practice statement affirms that all patients with KD should receive initial treatment with intravenous immunoglobulin (IVIG). In addition, we developed 7 strong and 4 conditional recommendations for the management of KD or suspected KD. Strong recommendations include prompt treatment of incomplete KD, treatment with aspirin, and obtaining an echocardiogram in the setting of unexplained macrophage activation syndrome or shock. Conditional recommendations include use of IVIG with other adjuvant agents for patients with KD and high-risk features of IVIG resistance and/or coronary artery aneurysms. These recommendations endorse minimizing risk to the patient by using established therapy promptly at disease onset and identifying situations in which adjunctive therapy may be warranted.
These recommendations provide guidance regarding diagnostic strategies, use of pharmacologic agents, and use of echocardiography in patients with suspected or confirmed KD.

To provide evidence-based recommendations and expert guidance for the management of Kawasaki disease (KD), focusing on clinical scenarios more commonly addressed by rheumatologists.
Sixteen clinical questions regarding diagnostic testing, treatment, and management of KD were developed in the Patient/Population, Intervention, Comparison, and Outcomes (PICO) question format. Systematic literature reviews were conducted for each PICO question. We used the Grading of Recommendations, Assessment, Development and Evaluation method to assess the quality of evidence and formulate recommendations. Each recommendation required consensus from at least 70% of the Voting Panel.
We present 1 good practice statement, 11 recommendations, and 1 ungraded position statement to guide the management of KD and clinical scenarios of suspected KD. These recommendations for KD are focused on situations in which input from rheumatologists may be requested by other managing specialists, such as in cases of treatment-refractory, severe, or complicated KD. The good practice statement affirms that all patients with KD should receive initial treatment with intravenous immunoglobulin (IVIG). In addition, we developed 7 strong and 4 conditional recommendations for the management of KD or suspected KD. Strong recommendations include prompt treatment of incomplete KD, treatment with aspirin, and obtaining an echocardiogram in the setting of unexplained macrophage activation syndrome or shock. Conditional recommendations include use of IVIG with other adjuvant agents for patients with KD and high-risk features of IVIG resistance and/or coronary artery aneurysms. These recommendations endorse minimizing risk to the patient by using established therapy promptly at disease onset and identifying situations in which adjunctive therapy may be warranted.
These recommendations provide guidance regarding diagnostic strategies, use of pharmacologic agents, and use of echocardiography in patients with suspected or confirmed KD.

川崎病为儿科临床常见的发热性疾病之一，中华医学会儿科学分会心血管学组、风湿学组、免疫学组及中华儿科杂志编辑委员会组织相关专家，制定了本共识，旨在更好地指导临床实践，提高我国川崎病的临床诊治水平，降低冠状动脉病变的发生率，改善患儿预后。.

Kawasaki disease, an acute systemic vasculitis affecting children, is the leading cause of acquired heart disease in developed countries. This vasculitis has a predilection for the coronary artery, and coronary artery abnormalities are the main criteria for its diagnosis. The diagnosis of coronary abnormalities has historically been based on dichotomous criteria, but recent guidelines have accepted the body surface area-adjusted z score system to define coronary abnormalities and classify coronary artery aneurysms. Z score systems have improved risk classifications of coronary aneurysms and improved correlations with clinical prognosis. However, the discrepancy of calculated z scores according to the formula has been noticed in the application of the z score system, which is possibly related to the diagnosis of coronary artery abnormalities. This variability was greater in larger coronary aneurysm dimensions. A careful choice of the z score formula and its consistent use is needed in clinical applications.

Kawasaki disease (KD), an acute, generalized vasculitis, is associated with an increased risk of coronary heart disease and is the most common cause of acquired heart disease in childhood. The incidence of KD is increasing worldwide. There are numerous international treatment guidelines. Our study aims to perform the first one so far comparison of them. While the gold standard therapy remains still the same (intravenous immunoglobulins and aspirin), there is currently a lack of evidence for choosing optimal treatment for high-risk patients and refractory KD. In this review, we also discuss the treatment of complications of KD and Kawasaki-like phenotypes, present an anti-inflammatory treatment in the light of new scientific data, and present novel potential therapeutic targets for KD.