Rheumatology Advance Access originally published online on December 20, 2005
Rheumatology 2006 45(2):240-241; doi:10.1093/rheumatology/kei071
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LETTER TO THE EDITOR |
Diagnosing Kawasaki syndrome
Department of Pediatrics, Children's Hospital, San Diego, CA and 1 Department of Behavioral Science and Health Education, Rollins School of Public Health, Emory University, Atlanta, GA, USA
Correspondence to: J. F. Bastian. E-mail: jbastian{at}chsd.org
SIR, We read with great interest the recent editorial on the need for a new tool for diagnosing Kawasaki syndrome/Kawasaki disease [1]. Our investigations of the history of the clinical criteria for Kawasaki syndrome [2–4] lead us to share a number of observations. Researchers have long noted that strict reliance on the classic case definition has led to delay in treatment of children who do not meet the classic criteria, yet still develop coronary artery aneurysms (CAA) [5]. Not only is the diagnosis delayed in these atypical cases, but recent North American studies report that the incidence of CAA is higher in the atypical cases than the classic ones [6, 7].
The goal of a clinical case definition of Kawasaki syndrome should be to prevent CAA. Although this may seem obvious, the classic case definition was formulated prior to the knowledge that Kawasaki syndrome can result in CAA [2, 3]. Simonini et al. [1] have reviewed their data and identified a combination of clinical signs and laboratory findings that may help identify patients with CAA who failed to meet the classic diagnostic criteria. As the authors note, the American Heart Association's Committee on Rheumatic Fever, Endocarditis, and Kawasaki Disease has recently issued an algorithm to better identify patients at risk of CAA [8]. Although these revised guidelines should help identify patients at risk of CAA who do not meet the classic criteria, they have not yet undergone prospective testing. We endorse Simonini et al.'s suggestion that any new guidelines undergo prospective analysis to determine their sensitivity and specificity for preventing CAA. In Japan, there has been prospective testing of the Harada scoring system, which relies on a combination of clinical and laboratory findings, for identification of patients with CAA [9]. Using the Harada criteria, Sato et al. [10] were able to successfully identify and treat those at risk of CAA in a study of 203 patients. Given its initial reliability, it seems that the Harada score is a reasonable starting point for the development of a clinical case definition to identify patients at risk of CAA.
Our other comments deal with phenotype and nosology. Because neither the agent(s) nor the pathophysiology that result in paediatric CAA are known, the illness should properly be labelled ‘Kawasaki syndrome’, not ‘Kawasaki disease’. The authors of the editorial and the Centers for Disease Control and Prevention have reverted to this nomenclature, but the American Heart Association and the Japan Kawasaki Disease Research Committee retain the term ‘Kawasaki disease’. As we have discussed in detail elsewhere [2, 3], the use of the term ‘Kawasaki disease’ came into the lexicon for political rather than scientific reasons. This distinction has important ramifications for both diagnosis and research. Only when an aetiological agent and/or pathophysiology are identified should the ‘disease’ nomenclature be adopted.
From this perspective, the adoption and use of the term ‘incomplete Kawasaki disease’ is misleading, because it presumes that all cases which develop CAA but fail to meet the classical criteria are anomalous. There are two reasons to resist such nomenclature. First, as a syndrome, the clinical signs, symptoms and laboratory findings (the phenotype) are necessarily tentative. Grouping classical and atypical presentations together as a syndrome authorizes epidemiologists and researchers to determine if these presentations comprise a single disease with a spectrum of possible outcomes or multiple diseases that share a number of common clinical presentations but have distinct causes and outcomes. The adoption of the term ‘incomplete’ assumes that the classical criteria represent and identify the essential elements that result in paediatric CAA. However, given the number of cases in which CAA develops despite the failure to meet the classical criteria, such a designation is both unhelpful and misleading. We believe that the terms ‘incomplete’ and ‘atypical’ should both be abandoned and the term ‘Kawasaki syndrome’ be adopted to denote illnesses that may result in paediatric CAA. The emphasis should be on the identification of those children at risk of CAA and not the preservation of a clinical sign complex.
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. [Editorial]. Rheumatology 2005; 44:959–61.
[Free Full Text] - Kushner HI, Bastian JF, Turner CL, Burns JC. Rethinking the boundaries of Kawasaki disease: toward the next case definition, Perspect Biol Med 2003:46:216–33.[Medline]
- Kushner HI, Turner CH, Bastian JF, Burns JC. The narratives of Kawasaki disease. Bull Hist Med 2004;78:410–39.[Medline]
- Kushner HI, Burns JC, Bastian JF, Turner CH. The histories of Kawasaki Disease. Progr Pediatr Cardiol 2004;19:91–7.[CrossRef]
- Rowley AH. Incomplete (atypical) Kawasaki disease. Pediatr Infect Dis J 2002;21:563–6.[CrossRef][ISI][Medline]
- Joffe A, Kabani A, Jadavji T. Atypical and complicated Kawasaki disease in infants: Do we need criteria? West J Med 1995;162:322–7.[ISI][Medline]
- Witt MT, Minich LL, Bohnsack JF, Young PC. Kawasaki disease: More patients are being diagnosed who do not meet the American Heart Association criteria. Pediatrics 1999;104:e10.
[Abstract/Free Full Text] - 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] - Harada K. Intravenous gamma-globulin treatment in Kawasaki disease. Acta Paediatr Jpn 1991;33:805–10.[Medline]
- Sato N, Sugimura T, Akagi T. 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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