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Incomplete (atypical) Kawasaki disease

INTRODUCTION

Kawasaki disease (KD, also called mucocutaneous lymph node syndrome) is one of the most common vasculitides of childhood [1]. KD also occurs rarely in adults. It is typically a self-limited condition, with fever and manifestations of acute inflammation lasting for an average of 11 days without therapy [2]. However, complications such as coronary artery aneurysms may develop and lead to significant morbidity and mortality.

Children suspected of having KD who do not fulfill diagnostic criteria (ie, have less than four signs of mucocutaneous inflammation) may have incomplete or atypical KD [1-3]. "Incomplete" KD is the preferred term, since these patients do not appear to differ from those with classic KD in any way except that they lack a sufficient number of criteria to fulfill the epidemiologic case definition [2]. Children with incomplete KD are also at risk for cardiovascular sequelae.

The clinical manifestations, diagnosis, and criteria for treatment of incomplete KD are discussed in this review. Unique features in infants and adults are also presented. Other aspects of Kawasaki disease are reviewed separately. (See "Kawasaki disease: Epidemiology and etiology" and "Kawasaki disease: Clinical features and diagnosis" and "Kawasaki disease: Initial treatment and prognosis" and "Kawasaki disease: Complications" and "Cardiovascular sequelae of Kawasaki disease".)

EPIDEMIOLOGY

The incidence of incomplete KD is unknown [4,5]. In a retrospective report of 242 Japanese children with KD treated at a single center over a nine-year period, 10 percent of patients were diagnosed with incomplete KD [4]. The incidence appears to be greater in infants younger than six months of age [5,6]. This was illustrated in a retrospective review of 44 children with KD; 5 of 11 infants (45 percent) had incomplete disease compared with 4 of 33 (12 percent) older children [5].

CLINICAL PRESENTATION OF TYPICAL VERSUS INCOMPLETE KD

Signs and symptoms appear to parallel those in children who fulfill diagnostic criteria for typical disease (table 1) when clinical judgment of reliable observers is used to define incomplete KD. (See "Kawasaki disease: Clinical features and diagnosis", section on 'Clinical manifestations' and 'Infants' below.)

        

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Literature review current through: Mar 2014. | This topic last updated: Jun 18, 2012.
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References
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  1. Burns JC, Glodé MP. Kawasaki syndrome. Lancet 2004; 364:533.
  2. 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. Circulation 2004; 110:2747.
  3. Cimaz R, Sundel R. Atypical and incomplete Kawasaki disease. Best Pract Res Clin Rheumatol 2009; 23:689.
  4. Fukushige J, Takahashi N, Ueda Y, Ueda K. Incidence and clinical features of incomplete Kawasaki disease. Acta Paediatr 1994; 83:1057.
  5. Joffe A, Kabani A, Jadavji T. Atypical and complicated Kawasaki disease in infants. Do we need criteria? West J Med 1995; 162:322.
  6. Rosenfeld EA, Corydon KE, Shulman ST. Kawasaki disease in infants less than one year of age. J Pediatr 1995; 126:524.
  7. Gomard-Mennesson E, Landron C, Dauphin C, et al. Kawasaki disease in adults: report of 10 cases. Medicine (Baltimore) 2010; 89:149.
  8. Bratincsak A, Reddy VD, Purohit PJ, et al. Coronary artery dilation in acute Kawasaki disease and acute illnesses associated with Fever. Pediatr Infect Dis J 2012; 31:924.
  9. Yellen ES, Gauvreau K, Takahashi M, et al. Performance of 2004 American Heart Association recommendations for treatment of Kawasaki disease. Pediatrics 2010; 125:e234.
  10. Levy M, Koren G. Atypical Kawasaki disease: analysis of clinical presentation and diagnostic clues. Pediatr Infect Dis J 1990; 9:122.
  11. Burns JC, Wiggins JW Jr, Toews WH, et al. Clinical spectrum of Kawasaki disease in infants younger than 6 months of age. J Pediatr 1986; 109:759.
  12. Cho MA, Choi YJ, Jung JW. Affects of "age at diagnosis" on coronary artery lesions in patients with incomplete kawasaki disease. Korean Circ J 2010; 40:283.
  13. Sonobe T, Kiyosawa N, Tsuchiya K, et al. Prevalence of coronary artery abnormality in incomplete Kawasaki disease. Pediatr Int 2007; 49:421.
  14. Sudo D, Monobe Y, Yashiro M, et al. Coronary artery lesions of incomplete Kawasaki disease: a nationwide survey in Japan. Eur J Pediatr 2012; 171:651.
  15. Yanagawa H, Tuohong Z, Oki I, et al. Effects of gamma-globulin on the cardiac sequelae of Kawasaki disease. Pediatr Cardiol 1999; 20:248.
  16. Burns JC, Capparelli EV, Brown JA, et al. Intravenous gamma-globulin treatment and retreatment in Kawasaki disease. US/Canadian Kawasaki Syndrome Study Group. Pediatr Infect Dis J 1998; 17:1144.
  17. Chang FY, Hwang B, Chen SJ, et al. Characteristics of Kawasaki disease in infants younger than six months of age. Pediatr Infect Dis J 2006; 25:241.
  18. Sbidian E, Lacert A, Perrin P, Le Cleach L. [Adult Kawasaki disease]. Ann Dermatol Venereol 2009; 136:260.
  19. Seeberg KA, Selbekk BH. [A man presenting with fever and abdominal pain]. Tidsskr Nor Laegeforen 2010; 130:1830.
  20. Sève P, Lega JC. [Kawasaki disease in adult patients]. Rev Med Interne 2011; 32:17.
  21. Everett ED. Mucocutaneous lymph node syndrome (Kawasaki disease) in adults. JAMA 1979; 242:542.
  22. Wolff AE, Hansen KE, Zakowski L. Acute Kawasaki disease: not just for kids. J Gen Intern Med 2007; 22:681.
  23. Cunha BA, Pherez FM, Alexiadis V, et al. Adult Kawasaki's disease with myocarditis, splenomegaly, and highly elevated serum ferritin levels. Heart Lung 2010; 39:164.