The 2021 American College of Rheumatology/Vasculitis Foundation guidelines for the management of Kawasaki disease (KD) provided evidence-based recommendations and expert guidance for the treatment of KD, with a focus on clinical situations more commonly handled by rheumatologists. The good practice statement confirmed that all KD patients should receive initial treatment with intravenous immunoglobulin (IVIg). Additionally, the guideline included 7 strong recommendations and 4 conditional recommendations for the treatment of KD or suspected KD.The strong recommendations emphasized the timely treatment of incomplete KD, the use of aspirin therapy, and the performance of echocardiography in cases of macrophage activation syndrome or shock of unknown origin. The conditional recommendations suggested the use of IVIg and other adjunctive therapies for patients with KD who were resistant to IVIg and/or had high-risk features for coronary artery aneurysms. This guideline provided direction on diagnostic strategies, drug selection, and the use of echocardiography for suspected or confirmed KD patients. This article referenced both domestic and international literature to interpret the key points of the guideline, aiming to provide useful insights for pediatricians in China.

1.Gorelik M, Chung SA, Ardalan K, et al. 2021 American College of Rheumatology/Vasculitis Foundation guideline for the management of Kawasaki disease[J]. Arthritis Rheumatol, 2022, 74(4): 586-596. DOI: 10.1002/art.42041.

2.McCrindle BW, Rowley AH, Newburger JW, et al. Diagnosis, treatment, and long-term management of Kawasaki disease: a scientific statement for health professionals from the American Heart Association[J].Circulation, 2017, 135(17): e927-e999. DOI: 10.1161/CIR.0000000000000484.

3.Burns JC. Frequently asked questions regarding treatment of Kawasaki disease[J]. Glob Cardiol Sci Pract, 2017(3): e201730. DOI: 10.21542/gcsp.2017.30.

4.黄玉娟, 李夙凌, 田园, 等. 中国儿童川崎病诊疗循证指南（2023年）[J]. 中国当代儿科杂志, 2023, 25(12): 1198-1210. [Huang YJ, Li SL, Tian Y, et al. Evidence-based guidelines for the diagnosis and treatment of Kawasaki disease in children in China (2023)[J]. Chinese Journal of Contemporary Pediatrics, 2023, 25(12): 1198-1210.] DOI: 10.7499/j.issn.1008-8830.2309038.

5.Marchesi A, Tarissi de Jacobis I, Rigante D, et al. Kawasaki disease: guidelines of Italian Society of Pediatrics, part II-treatment of resistant forms and cardiovascular complications, follow-up, lifestyle and prevention of cardiovascular risks[J]. Ital J Pediatr, 2018, 44(1): 103. DOI: 10.1186/s13052-018-0529-2.

6.Marchesi A, Tarissi de Jacobis I, Rigante D, et al. Kawasaki disease: guidelines of the Italian Society of Pediatrics, part I-definition, epidemiology, etiopathogenesis, clinical expression and management of the acute phase[J]. Ital J Pediatr, 2018, 44(1): 102. DOI: 10.1186/s13052-018-0536-3.

7.Kobayashi T, Ayusawa M, Suzuki H, et al. Revision of diagnostic guidelines for Kawasaki disease (6th revised edition)[J]. Pediatr Int, 2020, 62(10): 1135-1138. DOI: 10.1111/ped.14326.

8.de Graef N, Groot N, Ozen S, et al. European consensus-based recommendations for the diagnosis and treatment of Kawasaki disease-the SHARE initiative[J]. Rheumatology (Oxford), 2019, 58(4): 672-682. DOI: 10.1093/rheumatology/key344.

9.Newburger JW, Sleeper LA, McCrindle BW, et al. Randomized trial of pulsed corticosteroid therapy for primary treatment of Kawasaki disease[J]. N Engl J Med, 2007, 356(7): 663-675. DOI: 10.1056/NEJMoa061235.

10.Newburger JW, Takahashi M, Beiser AS, et al. A single intravenous infusion of gamma globulin as compared with four infusions in the treatment of acute Kawasaki syndrome[J]. N Engl J Med, 1991, 324(23): 1633-1639. DOI: 10.1056/NEJM199106063242305.

11.Kobayashi T, Saji T, Otani T, et al. Efficacy of immunoglobulin plus prednisolone for prevention of coronary artery abnormalities in severe Kawasaki disease (RAISE study): a randomised, open-label, blinded-endpoints trial[J]. Lancet, 2012, 379(9826): 1613-1620. DOI: 10.1016/S0140-6736(11)61930-2.

12.Son MBF, Gauvreau K, Tremoulet AH, et al. Risk model development and validation for prediction of coronary artery aneurysms in Kawasaki disease in a north American population[J]. J Am Heart Assoc, 2019, 8(11): e011319. DOI: 10.1161/JAHA.118.011319.

13.Dionne A, Burns JC, Dahdah N, et al. Treatment intensification in patients with Kawasaki disease and coronary aneurysm at diagnosis[J]. Pediatrics, 2019, 143(6): e20183341. DOI: 10.1542/peds.2018-3341.

14.刘芳, 林怡翔. 川崎病严重冠状动脉病变的长期管理 [J]. 临床儿科杂志, 2023, 41(7): 492-497. [Liu  F, Lin YX. Long-term management of severe coronary artery lesions in Kawasaki disease[J]. Journal of Clinical Pediatrics, 2023, 41(7): 492-497.] DOI: 10.12372/jcp. 2022.23e0331.

15.吴谨志, 闵丽, 董湘玉. 白细胞介素1及其受体拮抗剂阿那白滞素在川崎病中的研究进展[J]. 中国医药. 2021, 16(11): 1735-1739. [Wu JZ, Min L, Dong XY. Research progress on interleukin-1 and its receptor antagonist anakinra in Kawasaki disease[J]. China Medicine, 2021, 16(11): 1735-1739.] DOI: 10.3760/j.issn. 1673-4777.2021.11.031.

16.焦富勇, 穆志龙, 杜忠东, 等. 儿童不完全性川崎病的诊治[J]. 中国当代儿科杂志, 2023, 25(3): 238-243. [Jiao FY, Mu ZL, Du ZD, et al. Diagnosis and treatment of incomplete Kawasaki disease in children[J]. Chinese Journal of Contemporary Pediatrics, 2023, 25(3): 238-243.] DOI: 10.7499/j.issn.1008-8830.2209127.

17.Hu YC, Liu HM, Lin MT, et al. Outcomes of Kawasaki disease children with spontaneous defervescence within 10 days[J]. Front Pediatr, 2019, 7: 158. DOI: 10.3389/fped.2019.00158.

18.Wang W, Gong F, Zhu W, et al. Macrophage activation syndrome in Kawasaki disease: more common than we thought?[J]. Semin Arthritis Rheum, 2015, 44: 405-410. DOI: 10.1016/j.semarthrit.2014.07.007.

19.郝胜, 黄敏. 儿童川崎病相关巨噬细胞活化综合征的临床诊治[J]. 临床儿科杂志, 2023, 41(7): 486-491. [Hao  S, Huang M. Clinical diagnosis and treatment of Kawasaki disease-associated macrophage activation syndrome in children[J]. Journal of Clinical Pediatrics, 2023, 41(7): 486-491.] DOI: 10.12372/jcp.2022.23e0317.

20.Son MBF, Gauvreau K, Kim S, et al. Predicting coronary artery aneurysms in Kawasaki disease at a north American center: an assessment of baseline z scores[J]. J Am Heart Assoc, 2017, 6(6): e005378. DOI: 10.1161/JAHA.116.005378.

21.陕西省川崎病诊疗中心, 陕西省儿童内科疾病临床医学研究中心, 陕西省人民医院儿童病院, 等. 静脉输注免疫球蛋白在儿童川崎病中应用的专家共识 [J]. 中国当代儿科杂志, 2021: 23(9): 867-876. [Shaanxi Provincial Diagnosis and Treatment Center of Kawasaki Disease, Clinical Research Center for Childhood Diseases of Shaanxi Province, Children's Hospital of Shaanxi Provincial People's Hospital, et al. Expert consensus on the use of intravenous immunoglobulin in children with Kawasaki disease[J]. Chinese Journal of Contemporary Pediatrics, 2021, 23(9): 867-876. DOI: 10.7499/j.issn. 1008-8830.2107110.

22.Pendergrast J, Armali C, Callum J, et al. A prospective observational study of the incidence, natural history, and risk factors for intravenous immunoglobulin-mediated hemolysis[J]. Transfusion, 2021, 61(4): 1053-1063. DOI: 10.1111/trf.16232.

23.杜忠东, 谢利剑, 刘世平, 等. 阿司匹林在川崎病治疗中的儿科专家共识[J]. 中国当代儿科杂志, 2022, 24(6): 597-603. [Du ZD, Xie LJ, Liu SP, et al. Pediatric expert consensus on the application of aspirin in Kawasaki disease[J]. Chinese Journal of Contemporary Pediatrics, 2022, 24(6): 597-603.] DOI: 10.7499/j.issn.1008-8830. 2203190.

24.Koren G, Lavi S, Rose V, et al. Kawasaki disease: review of risk factors for coronary aneurysms[J]. J Pediatr, 1986, 108(3): 388-392. DOI: 10.1016/s0022-3476(86)80878-2.

25.Martins A, Conde M, Brito M, et al. Arthritis in Kawasaki disease: a poorly recognised manifestation[J]. J Paediatr Child Health, 2018, 54(12): 1371-1374. DOI: 10.1111/jpc.14102.