Rheumatology Advance Access originally published online on December 20, 2005
Rheumatology 2006 45(2):241; doi:10.1093/rheumatology/kei073
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LETTER TO THE EDITOR |
Reply: Nomenclature of Kawasaki disease/syndrome
Department of Paediatrics, Rheumatology Unit, 1 Department of Paediatrics, University of Firenze, Italy and 2 Division of Rheumatology, Department of Pediatrics, A. I. duPont Hospital for Children, Wilmington, DE and Department of Pediatrics, Thomas Jefferson University, Philadelphia, PA, USA
Correspondence to: G. Simonini. E-mail: gabriele.simonini{at}unifi.it
We thank Drs Bastian and Kushner for their helpful comments and suggestions on our editorial on Kawasaki syndrome [1].
We agree with their suggestion that the term Kawasaki syndrome (KS) is more appropriate than Kawasaki disease until we have a better understanding of the exact aetiology and pathogenesis of this vasculitic syndrome. Drs Bastian and Kushner state that ‘the goal of a KS clinical case definition should be to prevent coronary artery aneurysm. In our view this goal can be accomplished by developing a broader definition of the syndrome to ensure identification of all children who would benefit from iv Ig treatment’ [1].
Modifying case definition and classification criteria will not help. The terms ‘atypical’ and ‘incomplete’ are introduced to modify and supersede a rigid rule requiring a categorical answer!
The current criterion for diagnosing KS is based on case definition criteria created for epidemiological surveys [2, 3]. In many respects case definition has the same weakness as classification criteria. These tools work well for groups of patients, but not in the evaluation of individual patients [4]. Also, application of case definition criteria and classification criteria results in ‘yes’ or ‘no’ answers only. Therefore they function poorly in diagnosing individual patients.
Unfortunately, physicians often use case definition and classification criteria for diagnostic purposes. Since the complications of untreated KS can be fatal and since this can be greatly reduced with timely treatment, there is an urgent need for the physician to diagnose this condition early. However, the disease evolves over a few days to weeks and mimics several other common childhood diseases. Therefore, they need a different set of criteria capable of minimizing over- and underdiagnosis. Such diagnostic criteria should be able to exclude other diseases as much as possible (specificity) but, even more importantly, to include patients with probable KS (sensitivity). That is why we have been working on a more comprehensive set of diagnostic criteria that will group and label patients as having definite or probable KS.
Drs Bastian and Kushner also suggest that the Harada score may be a good start for developing a clinical case definition. We have already outlined our objection to using case definition for diagnostic purposes. Also, in the study by Sato and colleagues [5] referred to by Drs Bastian and Kushner, the Harada score was applied after patients had been diagnosed as having KS based on case definition criteria. Also, other than the age and sex of the patients, all the other items included in the score are laboratory values. We believe that any new diagnostic criteria should include a spectrum of clinical and laboratory values.
The authors have declared no conflicts of interest.
References
- Simonini G, Rose CD, Vierucci A, Falcini F, Athreya BH. Diagnosing Kawasaki syndrome: the need for a new clinical tool. Rheumatology 2005, Epub ahead of print 1 March 2005.
- Burns JC, Glode MP. Kawasaki Syndrome. Lancet 2004;364:533–44.[CrossRef][ISI][Medline]
- 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. Pediatrics 2004;114:1708–33.
[Abstract/Free Full Text] - Hunder GG. The use and misuse of classification and diagnostic criteria for complex diseases. Ann Intern Med 1998;129:417–8.
[Free Full Text] - Sato N, Sugimura T, Akagi T et al. Selective high dose gamma-globulin treatment in Kawasaki disease: assessment of clinical aspects and cost effectiveness. Pediatr Int 1999;41:1–7.[Medline]
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