Highlights & Basics
- Kawasaki disease is the second most common vasculitis in childhood (the most common being IgA vasculitis).
- Presents with an acute self-limiting febrile illness usually in young children, and is the leading cause of acquired heart disease in children ages under 5 years in the developed world.
- Classic features include fever, rash, conjunctivitis, lymphadenopathy, changes to the lips or oral mucosal, and peripheries, with initial swelling and erythema followed by desquamation in the second week.
- The most serious sequelae is coronary artery aneurysm, affecting 20% to 25% of untreated patients and significant proportions of treated patients in some countries, with even higher coronary artery aneurysm complication rates in those ages under 1 year.
- Current standard treatment includes intravenous immune globulin (IVIG), alongside aspirin. Adjunctive anti-inflammatory therapy (corticosteroids or tumor necrosis factor [TNF]-alpha inhibitors) should be considered early as part of initial therapy for high-risk patients and those with Kawasaki shock syndrome.
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McCrindle BW, Rowley AH, Newburger JW, et al; American Heart Association. Diagnosis, treatment, and long-term management of Kawasaki disease: a scientific statement for health professionals from the American Heart Association. Circulation. 2017 Apr 25;135(17):e927-99.[Abstract][Full Text]
de Graeff N, Groot N, Ozen S, et al. European consensus-based recommendations for the diagnosis and treatment of Kawasaki disease - the SHARE initiative. Rheumatology (Oxford). 2019 Apr 1;58(4):672-82.[Abstract][Full Text]
Jone PN, Tremoulet A, Choueiter N, et al. Update on diagnosis and management of Kawasaki disease: a scientific statement from the American Heart Association. Circulation. 2024 Dec 3;150(23):e481-500.[Abstract][Full Text]
Gorelik M, Chung SA, Ardalan K, et al. 2021 American College of Rheumatology/Vasculitis Foundation guideline for the management of Kawasaki disease. Arthritis Care Res (Hoboken). 2022 Apr;74(4):538-48.[Abstract]
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54. Tremoulet AH, Best BM, Song S, et al. Resistance to intravenous immunoglobulin in children with Kawasaki disease. J Pediatr. 2008 Jul;153(1):117-21.[Abstract][Full Text]
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65. 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. Lancet. 2012 Apr 28;379(9826):1613-20.[Abstract]
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