Multiple Outbreaks of Kawasaki Syndrome -- United States

Between August 22, 1984, and January 6, 1985, 10 outbreaks of Kawasaki syndrome (KS), a rare pediatric illness primarily affecting children under 6 years of age, were reported to CDC (Table 1). The outbreaks consisted of 187 cases meeting the CDC case definition* and 75 suspected cases. Outbreaks occurred in 10 states and the District of Columbia during the 21-week period. Cases from a number of these outbreaks continue to be reported to CDC.

Six of the 10 outbreaks occurred in major metropolitan areas: 39 (83%) of 47 cases in the Colorado outbreak occurred in the Denver metropolitan area, with the remaining eight extending south from Denver to Colorado Springs and north to Fort Collins, and to Cheyenne in southern Wyoming. Twenty (67%) of 30 cases in Massachusetts occurred in the Boston metropolitan area; all of 11 cases occurred in the Washington, D.C., metropolitan area; six (86%) of seven cases in Tennessee occurred in Memphis; 11 (92%) of 12 cases in California occurred in the Oakland/San Francisco metropolitan area; and nine of 10 cases in Texas occurred in the Houston metropolitan area. In Washington, all of 11 cases occurred in the nonmetropolitan areas of two adjacent counties (Pierce and King), and in North Carolina, cases were found in eight eastern counties. The Virginia and Indiana cases were widely scattered.

Patients' ages ranged from 7 weeks to 12 years 7 months (mean 2.6 years). Of 186 patients for whom sex was reported, 109 (59%) were males. For 177 patients for whom race was reported, 105 (59%) were white; 52 (29%), black; 16 (9%), Asian; and four (2%), Hispanic. A higher percentage of blacks was reported in Tennessee (six (86%) of seven) and eastern North Carolina (15 (56%) of 27); six of 12 California patients reported were of Asian extraction. Nationwide, 159 (85%) of 187 patients were hospitalized. Recurrent cases were reported in California (two cases) and North Carolina (one). To date, no outbreak-related fatalities have been reported.

Sixty-two (33%) of 186 patients had cardiovascular complications. Coronary artery aneurysms were reported in 37 (20%) cases; one resulted in myocardial infarction, and two were associated with pericarditis. Because coronary artery aneurysms are often not detected until 2-8 weeks after onset of KS, the number with this complication may increase. Myocarditis was reported in 12 patients, including one associated with a cardiac arrest, and another, with myocardial infarction and pericarditis. Eight additional patients had pericarditis. One child had recurrent angina episodes with a normal cardiac catheterization study; two had peripheral vascular complications resulting in gangrene and requiring amputations. One child had a stroke; and one had transient hemiparesis.

Noncardiovascular KS complications reported include: sterile pyuria/meatitis (14 cases), hydrops of the gallbladder (eight), hepatitis (six), arthritis (six), aseptic meningitis (four), uveitis (two), small bowel obstruction (one), and profound anemia requiring transfusion (one). Reported by M Glode, MD, The Children's Hospital, J Wiggins, MD, University of Colorado Medical Center, Denver, R Hopkins, MD, Communicable Disease Control, Colorado Dept of Health; P Pappas, MD, Wilmington, D Kredich, Duke University Medical Center, Durham, D Ingram, MD, R Warren, MD, University of North Carolina Medical Center, Chapel Hill, A Askew, MD, Raleigh, T McCutchen, Jr, MD, Fayetteville, N Patrone, MD, Greenville, J MacCormack, MD, State Epidemiologist, Div of Health Svcs, North Carolina Dept of Human Resources; L Branch, G Miller, Jr, MD, State Epidemiologist, Virginia State Dept of Health; R Wientzen, MD, Georgetown University Medical Center, B Wiedermann, MD, Children's Hospital National Medical Center, M Levy, MD, State Epidemiologist, District of Columbia Dept of Human Svcs; G Gallemore, MD, Quillen Dishner School of Medicine, Johnson City, F Barrett, MD, LeBonheur Hospital, Memphis, R Hutcheson, Jr, MD, State Epidemiologist, Tennessee State Dept of Health and Environment; J Burns, MD, J Newberger, MD, D Leung, MD, Boston Children's Hospital, D Medearis, MD, Dept of Pediatrics, Massachusetts General Hospital, D Fulton, MD, H Meissner, MD, Dept of Pediatrics, New England Medical Center, Boston, J Sullivan, MD, University of Massachusetts School of Medicine, Worcester, G Grady, MD, State Epidemiologist, Massachusetts Dept of Public Health; D Seavey, S Kaplan, MD, Texas Children's Hospital, Houston, C Alexander, MD, State Epidemiologist, Texas Dept of Health; E Southwood, M Kleiman, MD, James Whitcomb Riley Hospital for Children, Indianapolis, C Barrett, MD, State Epidemiologist, Indiana State Board of Health; J Kobayashi, MD, State Epidemiologist, Washington Dept of Social and Health Svcs; R Benjamin, MD, Alameda County Health Dept, J Chin, MD, State Epidemiologist, California Dept of Health Svcs; Div of Viral Diseases, Center for Infectious Diseases, CDC.

Editorial Note

Editorial Note: KS, first described by a Japanese pediatrician in 1961 (1), has been occurring in the United States since at least 1971 (2). Its etiology is unknown. Evidence for person-to-person transmission has not been demonstrated. In the United States, peaks of occurrence of KS have been observed in winter and spring. Although coronary artery aneurysms have been reported in 17%-31% of cases (3), fatalities are relatively rare. Case-fatality ratios of 1%-2% (4) have been reported in the United States and Japan.

Other complications of KS include pyuria and urethritis/meatitis, arthritis, aseptic meningitis, myocarditis, pericarditis, pericardial effusion, hepatitis, and hydrops of the gallbladder (5). Patients with gangrenous extremities, small bowel obstruction, and stroke have been very rarely reported.

The efficacy of any single therapeutic regimen has not been well established, although one study has suggested that the administration of aspirin during the acute phase (6), and another, that the administration of intravenous high-dose gammaglobulin during the acute phase (7), may reduce the frequency of coronary artery aneurysms. A multicenter study to evaluate the potential efficacy of high-dose intravenous gammaglobulin therapy is currently under way in the United States.

Physicians are encouraged to report any outbreaks or cases of KS through their 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 lymph node involvement with septic desquamation of the fingers and toes in children. Jpn J Allerg 1967;16:178-222.

2. Melish ME, Hicks RM, Larson EJ. Mucocutaneous lymph node syndrome in the United States. Am J Dis Child, 1976;130:599-607.

3. 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.

4. Morens DM, Anderson LJ, Hurwitz ES. National surveillance of Kawasaki disease. Pediatrics 1980; 65:21-5.

5. CDC. Unpublished data.

6. Kato H, Koike S, Yokoyama T. Kawasaki disease: effect of treatment on coronary artery involvement. Pediatrics 1979;63:175-9.

7. Furusho K, Sato K, Soeda T, et al. High-dose intravenous gammaglobulin for Kawasaki disease (Letter). Lancet 1983;II:1359. *Fever lasting 5 or more days without other more reasonable explanation and 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 lymph node 1.5 cm or greater in diameter).

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