Evidence-based guidelines for the diagnosis and treatment of Kawasaki disease in children in China (2023)
Center for Diagnosis and Treatment of Kawasaki Disease/Children's Hospital of Shaanxi Provincial People's Hospital, National Children's Medical Center/Beijing Children's Hospital, Capital Medical University, Children's Hospital, Shanghai Jiao Tong University School of Medicine, National Regional Medical Center/Shengjing Hospital of China Medical University, National Clinical Key Specialty/Department of Intensive Care Medicine, Shanghai Children's Hospital, General Pediatric Group of Pediatrician Branch of Chinese Medical Doctor Association, Expert Committee of Advanced Training for Pediatrician, China Maternal and Children's Health Association, National Health Commission Key Laboratory for Tropical Disease Prevention and Control, Yan'an University Affiliated Hospital, Editorial Department of Chinese Journal of Contemporary Pediatrics
Abstract: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.
Kawasaki T. Acute febrile mucocutaneous syndrome with lymphoid involvement with specific desquamation of the fingers and toes in children[J]. Arerugi, 1967, 16(3): 178-222. PMID: 6062087.
Chen Y, Yang K, Maru?ic A, et al. A reporting tool for practice guidelines in health care: the RIGHT statement[J]. Ann Intern Med, 2017, 166(2): 128-132. PMID: 27893062. DOI: 10.7326/M16-1565.
11 Institute of Medicine (U.S.). Committee on Standards for Developing Trustworthy Clinical Practice Guidelines. Clinical Practice Guidelines We Can Trust[M]. Washington, DC, USA: National Academies Press, 2011.
Lewin S, Glenton C, Munthe-Kaas H, et al. Using qualitative evidence in decision making for health and social interventions: an approach to assess confidence in findings from qualitative evidence syntheses (GRADE-CERQual)[J]. PLoS Med, 2015, 12(10): e1001895. PMID: 26506244. PMCID: PMC4624425. DOI: 10.1371/journal.pmed.1001895.
Le CY, Truong LK, Holt CJ, et al. Searching for the holy grail: a systematic review of health-related quality of life measures for active youth[J]. J Orthop Sports Phys Ther, 2021, 51(10): 478-491. PMID: 34592831. DOI: 10.2519/jospt.2021.10412.
Guyatt GH, Alonso-Coello P, Schünemann HJ, et al. Guideline panels should seldom make good practice statements: guidance from the GRADE working group[J]. J Clin Epidemiol, 2016, 80: 3-7. PMID: 27452192. DOI: 10.1016/j.jclinepi.2016.07.006.
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. PMID: 28356445. DOI: 10.1161/CIR.0000000000000484.
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. PMID: 33001522. DOI: 10.1111/ped.14326.
de Graeff 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. PMID: 30535127. DOI: 10.1093/rheumatology/key344.
Gorelik M, Chung SA, Ardalan K, et al. 2021 American College of Rheumatology/Vasculitis Foundation guideline for the management of Kawasaki disease[J]. Arthritis Care Res (Hoboken), 2022, 74(4): 586-596. PMID: 35257501. DOI: 10.1002/art.42041.
Phuong LK, Bonetto C, Buttery J, et al. Kawasaki disease and immunisation: standardised case definition & guidelines for data collection, analysis[J]. Vaccine, 2016, 34(51): 6582-6596. PMID: 27863715. DOI: 10.1016/j.vaccine.2016.09.025.
Fukazawa R, Kobayashi J, Ayusawa M, et al. JCS/JSCS 2020 guideline on diagnosis and management of cardiovascular sequelae in Kawasaki disease[J]. Circ J, 2020, 84(8): 1348-1407. PMID: 32641591. DOI: 10.1253/circj.CJ-19-1094.
Ha KS, Jang G, Lee J, et al. Incomplete clinical manifestation as a risk factor for coronary artery abnormalities in Kawasaki disease: a meta-analysis[J]. Eur J Pediatr, 2013, 172(3): 343-349. PMID: 23229186. DOI: 10.1007/s00431-012-1891-5.
Alphonse MP, Duong TT, Shumitzu C, et al. Inositol-triphosphate 3-Kinase C mediates inflammasome activation and treatment response in Kawasaki disease[J]. J Immunol, 2016, 197(9): 3481-3489. PMID: 27694492. DOI: 10.4049/jimmunol.1600388.
Anzai F, Watanabe S, Kimura H, et al. Crucial role of NLRP3 inflammasome in a murine model of Kawasaki disease[J]. J Mol Cell Cardiol, 2020, 138: 185-196. PMID: 31836541. DOI: 10.1016/j.yjmcc.2019.11.158.
Farias JS, Villarreal EG, Savorgnan F, et al. The use of neutrophil-lymphocyte ratio for the prediction of refractory disease and coronary artery lesions in patients with Kawasaki disease[J]. Cardiol Young, 2023, 33(8): 1409-1417. PMID: 37012661. DOI: 10.1017/S1047951123000653.
Research Committee of the Japanese Society of Pediatric Cardiology, Cardiac Surgery Committee for Development of Guidelines for Medical Treatment of Acute Kawasaki Disease. Guidelines for medical treatment of acute Kawasaki disease: report of the Research Committee of the Japanese Society of Pediatric Cardiology and Cardiac Surgery (2012 revised version)[J]. Pediatr Int, 2014, 56(2): 135-158. PMID: 24730626. DOI: 10.1111/ped.12317.
Newburger JW, Takahashi M, Gerber MA, et al. Diagnosis, treatment, and long-term management of Kawasaki disease: a statement for health professionals from the committee on Rheumatic Fever, Endocarditis, and Kawasaki Disease, Council on Cardiovascular Disease in the Young, American Heart Association[J]. Pediatrics, 2004, 114(6): 1708-1733. PMID: 15574639. DOI: 10.1542/peds.2004-2182.
Kim GB, Yu JJ, Yoon KL, et al. Medium- or higher-dose acetylsalicylic acid for acute Kawasaki disease and patient outcomes[J]. J Pediatr, 2017, 184: 125-129.e1. PMID: 28043685. DOI: 10.1016/j.jpeds.2016.12.019.
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. PMID: 31130036. PMCID: PMC6585355. DOI: 10.1161/JAHA.118.011319.
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. PMID: 30157897. PMCID: PMC6116535. DOI: 10.1186/s13052-018-0536-3.
Muta H, Ishii M, Yashiro M, et al. Late intravenous immunoglobulin treatment in patients with Kawasaki disease[J]. Pediatrics, 2012, 129(2): e291-e297. PMID: 22250032. DOI: 10.1542/peds.2011-1704.
Roberts SC, Jain S, Tremoulet AH, et al. The Kawasaki disease comparative effectiveness (KIDCARE) trial: a phase III, randomized trial of second intravenous immunoglobulin versus infliximab for resistant Kawasaki disease[J]. Contemp Clin Trials, 2019, 79: 98-103. PMID: 30840903. DOI: 10.1016/j.cct.2019.02.008.
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. PMID: 31048414. DOI: 10.1542/peds.2018-3341.
Kobayashi T, Kobayashi T, Morikawa A, et al. Efficacy of intravenous immunoglobulin combined with prednisolone following resistance to initial intravenous immunoglobulin treatment of acute Kawasaki disease[J]. J Pediatr, 2013, 163(2): 521-526. PMID: 23485027. DOI: 10.1016/j.jpeds.2013.01.022.
Burns JC, Roberts SC, Tremoulet AH, et al. Infliximab versus second intravenous immunoglobulin for treatment of resistant Kawasaki disease in the USA (KIDCARE): a randomised, multicentre comparative effectiveness trial[J]. Lancet Child Adolesc Health, 2021, 5(12): 852-861. PMID: 34715057. DOI: 10.1016/S2352-4642(21)00270-4.
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. PMID: 33433931. DOI: 10.1111/trf.16232.
Okubo Y, Miura M, Kobayashi T, et al. The impact of changes in clinical guideline on practice patterns and healthcare utilizations for Kawasaki disease in Japan[J]. Front Pediatr, 2020, 8: 114. PMID: 32266191. PMCID: PMC7105796. DOI: 10.3389/fped.2020.00114.
Kabbaha S, Milano A, Aldeyab MA, et al. Infliximab as a second-line therapy for children with refractory Kawasaki disease: a systematic review and meta-analysis of randomized controlled trials[J]. Br J Clin Pharmacol, 2023, 89(1): 49-60. PMID: 36169097. DOI: 10.1111/bcp.15547.
Brogan PA, Bose A, Burgner D, et al. Kawasaki disease: an evidence based approach to diagnosis, treatment, and proposals for future research[J]. Arch Dis Child, 2002, 86(4): 286-290. PMID: 11919108. PMCID: PMC1719139. DOI: 10.1136/adc.86.4.286.
Wilson N, Heaton P, Calder L, et al. Kawasaki disease with severe cardiac sequelae: lessons from recent New Zealand experience[J]. J Paediatr Child Health, 2004, 40(9-10): 524-529. PMID: 15367145. DOI: 10.1111/j.1440-1754.2004.00456.x.
Lee KY, Oh JH, Han JW, et al. Arthritis in Kawasaki disease after responding to intravenous immunoglobulin treatment[J]. Eur J Pediatr, 2005, 164(7): 451-452. PMID: 15782294. DOI: 10.1007/s00431-005-1653-8.
Suzuki A, Miyagawa-Tomita S, Komatsu K, et al. Immunohistochemical study of apparently intact coronary artery in a child after Kawasaki disease[J]. Pediatr Int, 2004, 46(5): 590-596. PMID: 15491390. DOI: 10.1111/j.1442-200x.2004.01943.x.
Taddio A, Rossi ED, Monasta L, et al. Describing Kawasaki shock syndrome: results from a retrospective study and literature review[J]. Clin Rheumatol, 2017, 36(1): 223-228. PMID: 27230223. DOI: 10.1007/s10067-016-3316-8.
Li Y, Zheng Q, Zou L, et al. Kawasaki disease shock syndrome: clinical characteristics and possible use of IL-6, IL-10 and IFN-γ as biomarkers for early recognition[J]. Pediatr Rheumatol Online J, 2019, 17(1): 1. PMID: 30611297. PMCID: PMC6321686. DOI: 10.1186/s12969-018-0303-4.
Harrison M, Scalici P. Clinical guideline highlights for the hospitalist: management of Kawasaki disease[J]. J Hosp Med, 2022, 17(10): 829-831. PMID: 35934950. DOI: 10.1002/jhm.12935.
Tremoulet AH, Jain S, Jaggi P, et al. Infliximab for intensification of primary therapy for Kawasaki disease: a phase 3 randomised, double-blind, placebo-controlled trial[J]. Lancet, 2014, 383(9930): 1731-1738. PMID: 24572997. DOI: 10.1016/S0140-6736(13)62298-9.
Latino GA, Manlhiot C, Yeung RS, et al. Macrophage activation syndrome in the acute phase of Kawasaki disease[J]. J Pediatr Hematol Oncol, 2010, 32(7): 527-531. PMID: 20485197. DOI: 10.1097/MPH.0b013e3181dccbf4.
Fukazawa R, Kobayashi T, Mikami M, et al. Nationwide survey of patients with giant coronary aneurysm secondary to Kawasaki disease 1999-2010 in Japan[J]. Circ J, 2017, 82(1): 239-246. PMID: 28855435. DOI: 10.1253/circj.CJ-17-0433.
La Rosée P, Horne A, Hines M, et al. Recommendations for the management of hemophagocytic lymphohistiocytosis in adults[J]. Blood, 2019, 133(23): 2465-2477. PMID: 30992265. DOI: 10.1182/blood.2018894618.