BMJ 2003;327:917 (18 October), doi:10.1136/bmj.327.7420.917
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Commentary
diagnosis is based on clinical features
David Burgner, paediatric infectious diseases physician1
1 School of Paediatrics and Child Health, University of Western Australia, Perth, Australia [email protected]
Kawasaki disease is a not uncommon and probably underdiagnosed paediatric vasculitis. The incidence of the disease in the United Kingdom, as in other countries, seems to be increasing.1 Kawasaki disease often enters the differential of febrile childhood illnesses, and this case shows the difficulties of making a timely diagnosis. Early recognition is critical; Kawasaki disease is the commonest cause of acquired heart disease in children, with about one third of untreated children developing coronary artery damage with a short term increased risk of death and serious long term sequelae. Prompt treatment within the first 10 days prevents overt coronary damage in most cases.2
The cause of Kawasaki disease is unknown, and consequently there is no diagnostic test. The diagnosis is made on a cluster of clinical signs (box). The diagnostic criteria are specific but lack sensitivity; atypical Kawasaki disease, in which coronary damage occurs without full diagnostic criteria is well recognised, especially in children outside the typical age range of 6 months to 5 years.3 The prolonged fever differentiates Kawasaki disease from many self limiting childhood infections, which it may resemble in the early stages. The other diagnostic features, as illustrated in this case, may present sequentially or transiently and need to be sought specifically. Lymphadenopathy is the least common finding, occurring in about 75% of cases; other features occur in well over 90%. Other diagnostically discriminating features include extreme irritability, much more than seen in other febrile illnesses, and inflammation of a recent BCG scar.
Clinicians often focus on the thrombocytosis and peripheral skin peeling to diagnose Kawasaki disease. However, both features occur in the convalescent phase when the coronary aneurysms develop and should never be relied on to aid the diagnosis. Perineal skin peeling, as in this case, may occur acutely.
Despite treatment with immunoglobulin and aspirin, subtle changes to the coronary vessels are reported in children who were thought to have escaped coronary lesions acutely.4 Such changes may have long term implications for cardiovascular health by predisposing to atherosclerosis,5 although definitive follow up studies are lacking.
Diagnostic criteria for Kawasaki disease2
Fever for over 5 days plus at least four of the following criteria:
Bulbar non-exudative conjunctivitis (figure)
Polymorphous rash
Changes in the extremities (initially erythema and oedema, subsequent periungual desquamation)
Changes to the lips or oral mucosa
Cervical lymphadenopathy
Non-exudative conjunctivitis
Credit: JKI
What causes Kawasaki disease?
Much research has focused on the pursuit of a single causative infectious agent. However, although Kawasaki disease is clearly infectious in origin, it is more likely to be initiated by one or more widely distributed pathogens that incite an abnormal inflammatory response in genetically susceptible individuals. Investigation of host genetics may therefore help understand the determinants of both susceptibility and coronary damage.
In adult cardiovascular disease, inflammation and the host response are increasingly recognised as critical factors in atherosclerosis.6 This inflammation may be induced by infection; cardiovascular risk correlates with the burden of infectious diseases.7 Kawasaki disease may therefore represent an extreme phenotype of a much more common but largely unrecognised phenomenon—seemingly trivial infections occurring early in life in genetically susceptible individuals paving the way to subsequent adult cardiovascular disease.
Harnden A, Alves B, Sheikh A. Rising incidence of Kawasaki disease in England: analysis of hospital admission data. BMJ 2002;324: 1424-5.[Free Full Text]
Brogan PA, Bose A, Burgner D, Shingadia D, Tulloh R, Michie C, et al. Kawasaki disease: an evidence based approach to diagnosis, treatment, and proposals for future research. Arch Dis Child 2002;86: 286-90.[Abstract/Free Full Text]
Rowley AH. Incomplete (atypical) Kawasaki disease. Pediatr Infect Dis J 2002;21: 563-5.[CrossRef][ISI][Medline]
Naoe S, Takahashi K, Masuda H, Tanaka N. Coronary findings post Kawasaki disease in children who died of other causes. Prog Clin Biol Res 1987;250: 341-6.[Medline]
Takahashi K, Oharaseki T, Naoe S. Pathological study of postcoronary arteritis in adolescents and young adults: with reference to the relationship between sequelae of Kawasaki disease and atherosclerosis. Pediatr Cardiol 2001;22: 138-42.[CrossRef][ISI][Medline]
De Boer OJ, van der Wal AC, Becker AE. Atherosclerosis, inflammation, and infection. J Pathol 2000;190: 237-43.[CrossRef][ISI][Medline]
Espinola-Klein C, Rupprecht HJ, Blankenberg S, Bickel C, Kopp H, Rippin G, et al. Impact of infectious burden on extent and long-term prognosis of atherosclerosis. Circulation 2002;105: 15-21.[Abstract/Free Full Text]
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A 2 year old child with rash and fever
Rita Sharma, Andrew Boon, and Anthony Harnden
BMJ 2003 327: 916. [Extract] [Full Text]
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