Kawasaki Syndrome -- United States

Since October 1982, four outbreaks of Kawasaki syndrome (KS), a rare illness affecting primarily preschool-aged children, have been reported to CDC. The outbreaks consisted of 43 cases in four states over a 3-month period. There were no fatalities.

Illinois: Eleven cases occurred in four contiguous counties--Cook, Will, DuPage, and McHenry--in December and January. The mean age was 4.3 years (range 11 months-12 years); four were female; 10 were white; one was black. Eight were hospitalized. Two reportedly had coronary aneurysms.

Michigan: Nine children with onset of KS between November 3 and December 31, 1982, were reported in Kent County (Grand Rapids). Their mean age was 3.1 years (range 8 months-8 years 5 months); seven were female; six were white; three were black. Eight of the nine were hospitalized. Illness in one child was complicated by carditis, pericardial effusion, and mild ST segment elevation. Scattered cases have been reported elsewhere in the state.

New York: Seven cases with onset between October 15, 1982, and January 20, 1983, were reported in two adjoining counties, Oneida and Herkimer. The mean age was 2.6 years (range 12 months-6 years 1 month); four cases occurred in females; all were white. Six of the seven were hospitalized. One developed coronary artery aneurysms.

Wisconsin: Sixteen cases occurred between November 28, 1982, and February 3, 1983, in Milwaukee and four adjoining counties. The mean age was 2.3 years (range 8 months-6 years 4 months); eight of the children were female; 12 were white; two were black; two were Asian. All 16 were hospitalized. The echocardiograms of eight children were abnormal, showing tubular dilatation of the right and left coronary arteries in one child, mild left heart enlargement in five children, and minimal posterior pericardial effusion in two children. Reported by A Baker, MD, Oneida County Health Dept, R Maggiolino, R Rothenberg, MD, State Epidemiologist, New York State Dept of Health; S Gardner, S Shulman, MD, Children's Memorial Hospital, Northwestern University Medical School, Chicago, KT Reddi, MD, Chicago Board of Health, BJ Francis, MD, State Epidemiologist, Illinois State Dept of Public Health; D Mack, MD, C Jillson, Kent County Health Dept, WN Hall, MD, KR Wilcox, Jr, MD, State Epidemiologist, Michigan State Dept of Public Health; DW Wortman, MD, B Mohr, Milwaukee Children's Hospital, JP Davis, MD, State Epidemiologist, Wisconsin Div of Health; Div of Viral Diseases, Center for Infectious Diseases, CDC.

Editorial Note

Editorial Note: KS, first described by a Japanese pediatrician in 1967, has been occurring in the United States since at least 1971 (1). The etiology is unknown. The diagnosis is clinical, and by CDC definition, a KS patient must have fever lasting 5 or more days without other reasonable explanation and satisfy at least four of the following criteria: 1) bilateral conjunctival injection; 2) at least one of the following mucous membrane changes: injected or fissured lips, injected pharynx, or "strawberry" tongue; 3) at least one of the following extremity changes: erythema of palms or soles, edema of the hands or feet, or generalized or periungual desquamation; 4) rash; and 5) cervical lymphadenopathy (at least one node 1.5 cm or larger in diameter). Coronary artery aneurysms are present in 17%-31% of cases (2), and the case-fatality ratio is approximately 1% (3).

KS outbreaks have been reported previously in the New York City area (4), Monroe County, New York (5), Los Angeles, California (6), eastern Massachusetts (5), Hawaii (7), and Denver, Colorado (8). These outbreaks, as well as cases reported to CDC through the national surveillance system (9), tend to occur during winter and spring.

Investigation of the Monroe County and eastern Massachusetts outbreaks revealed that children with KS had a higher incidence of an antecedent, primarily respiratory, illness (during the 30 days before onset of KS) than did controls matched for age, sex, and race. Neither serologic nor isolation studies incriminated a single etiologic agent for this antecedent illness. This investigation also revealed that KS was more likely to occur in children of middle and upper socioeconomic status.

A recently reported case-control study conducted during the Denver outbreak revealed that 11 (48%) of 23 case families, compared with nine (10%) of 86 control families, had shampooed their carpets within 30 days before onset of illness. Ten of these case parents allowed their children on the newly shampooed carpets within 2 hours, whereas control parents who shampooed tended to keep children off the carpets for 4 or more hours. The interval between shampooing and illness for nine of the 11 patients was 16-25 days.

Japanese investigators have proposed an association between house-dust mites (Dermatophagoides) and KS. In one study, serum levels of anti-mite-specific IgG in 15 of 20 KS patients were more than two SD above that found in 45 age-range-matched, non-atopic controls. Serum levels of anti-mite-specific IgE were similarly elevated in six of 20 KS patients. Mite antigen was found in circulating immune complexes in three of eight KS patients (10). This and another study (11) have not yet been repeated by other investigators, and their significance has yet to be determined.

In cooperation with CDC, state and local health departments in Michigan, New York, and Wisconsin are currently conducting outbreak investigations. Reports of any outbreaks or cases, or requests for further information, may be directed through local and state health departments to the Epidemiology Office, Division of Viral Diseases, Center for Infectious Diseases, CDC.

References

1. Kawasaki T. Acute febrile mucocutaneous syndrome with lymphoid involvement with specific desquamation of the fingers and toes in children. Jpn J Allerg 1967;16:178-222.

2. Kato H. Natural history of Kawasaki disease. In: Proceedings of the international workshop on vascular lesions of collagen diseases and related conditions. Tokyo: University of Tokyo Press, 1977:281-6.

3. Morens DM. Kawasaki disease (mucocutaneous lymph node syndrome). In: Feigin RD, Cherry JD, eds. Textbook of pediatric infectious diseases. Philadelphia: WB Saunders, 1981:1637-48.

4. CDC. Kawasaki disease--New York. MMWR 1980;29:61-3.

5. Bell DM, Brink EW, Nitzkin JL, et al. Kawasaki syndrome: description of two outbreaks in the United States. N Engl J Med 1981;304:1568-75.

6. CDC. Unpublished data.

7. Dean AG, Melish ME, Hicks R, Palumbo NE. An epidemic of Kawasaki syndrome in Hawaii. J Pediatr 1982;100:552-7.

8. Patriarca PA, Rogers MF, Morens DM, et al. Kawasaki syndrome: association with the application of rug shampoo. Lancet 1982;2:578-80.

9. Bell DM, Morens DM, Holman RC, Hurwitz ES. Kawasaki syndrome in the United States: 1976-1980. (In press: Am J Dis Child).

10. Furusho K, Ohba T, Soeda T, Kimoto K, Okabe T, Hirota T. Possible

11. Patriarca PA, Rogers MF, Morens DM, et al. Kawasaki syndrome: association with the application of rug shampoo. Lancet 1982;2:578-80.

12. Bell DM, Morens DM, Holman RC, Hurwitz ES. Kawasaki syndrome in the United States: 1976-1980. (In press: Am J Dis Child).

13. Furusho K, Ohba T, Soeda T, Kimoto K, Okabe T, Hirota T. Possible role for mite antigen in Kawasaki disease. Lancet 1981;2:194-5. Letter.

14. Hamashima Y, Tasaka K, Hoshino T, et al. Mite-associated particles in Kawasaki disease. Lancet 1982;2:266.

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