KAWASAKI DISEASE 1961-2005
Tomisaku Kawasaki *
Department of Pediatrics.*
I would like to give you a short history of Kawasaki Disease and I hope that it can be of reference for you.

After I had been practicing pediatrics for 10 years, in January 1961, I saw a curious clinical symptom complex case of a 4 years 3 months old boy. This is a slide of the clinical course of this case. High fever lasted for 2 weeks, there was bilateral conjunctival hyperemia, dried, reddish, cracked lips with diffuse erythema of the oral cavity, right cervical adenopathy, erythematous skin rash on the body, red palms and soles with indurative edema of the hands and feet. During his course, Combs positive hemolytic anemia and mild jaundice was also seen. Desquamation from the fingertips occurred. Laboratory findings showed that the erythrocyte sedimentation rates were remarkably elevated. C-reactive protein (CRP) was strongly positive and the blood test showed remarkable leucocytosis with shift to the left. I first suspected bacterial infection but there was no evidence of bacterial infection in the blood culture and throat culture. Treatment with antibiotics, including penicillin, had no effect on the child.

The nature of rash made me suspect allergic reaction, so prednisolone was administered but it also had no effect. After the fever had lasted for 2 weeks, it gradually declined. As antibiotics combined with steroids had no effect in this condition, I considered it a self-limiting illness.

I presented the case at a clinical conference where one pediatrician suggested that it could be a case of atypical scarlet fever. But the nature of the rash was different from scarlet fever, the throat culture was negative for A-group streptococci and penicillin therapy had no effect - so I replied that it was not Stevens-Johnson syndrome.

Another doctor suggested it was a mild case of Stevens-Johnson syndrome. But the rash had no vesicles, inside the mouth there was no ulcer formation and uro-genital area was free - so I replied that it was not Stevens-Johnson syndrome.

Finally, when the case was discharged, I decided it was "diagnosis unknown." I could not get the case out of my mind and always wondered what the diagnosis was.

A year later, in February 1962, when on night duty, a suspected case of sepsis was referred to us by a neighborhood doctor. When I was waiting in the emergency room, the mother entered carrying the baby and sat down in front of me. As soon as I saw the face of the baby, I was very surprised to see that it closely resembled the appearance of my case of the previous year. After admitting the case into our hospital and following the case very carefully, it followed almost the same course as the "diagnosis unknown" case, except for Coombs positive hemolytic anemia and jaundice. I then realized that I had seen two cases of a unique symptom complex which did not exist in any textbook.

In that year (i.e. 1962), I experienced 5 more similar cases. At a local pediatrics meeting I presented a paper entitled, "Non-scarlet fever syndrome with desquamation from the fingertips" and reported my 7 cases.
From 1963 onwards, every year I experienced several cases and in 6 years, I saw 50 cases. In 1967, I published an article entitled "Acute febrile mucocutaneous lymph node syndrome" in which I categorized the clinical symptoms, which as a complex were new. Inquiries from pediatricians all over Japan came to me and I realized there were similar cases being experienced all over the country.

In 1970, the Kawasaki Disease (KD) Research Committee sponsored by the Japanese Government was established. The KD Research Committee consisted of epidemiologists, pediatricians, microbiologists and pathologists. The first nation-wide epidemiologic survey was carried out in 1970. This Research Committee compiled the first edition of "Diagnostic Guidelines of KD (5th revised edition)". The guidelines were disseminated to all hospitals with more than 100 beds and having a separate pediatric department. A questionnaire was sent to these hospitals with the question of whether or not they had experienced cases according to the guidelines.

The Research Committee was able to gather information from around the country on 1100 cases, including more than 20 sudden-death cases. Among them, 10 cases were chosen and the doctors who experienced sudden-death cases were invited to come to Tokyo and attend a meeting for further discussion. At the meeting, it was found that the clinical features of all cases matched the guidelines. Out of 10 cases, 4 cases had been autopsied. All 4 cases showed right and left coronary artery aneurysms with thrombotic occlusion and the pathological diagnosis was Infantile Periarteritis Nodosa (IPN). At this time, the committee became aware that Kawasaki Disease was a kind of vasculitic syndrome.

Since then, 17 nation-wide surveys of KD have been carried out in Japan at two-year intervals and a total of 186,069 patients including 419 fatal cases (0.23%) were registered by the end of December 2002, the largest number throughout the world. Recently, according to a preliminary report of the 18th nation-wide epidemiological survey (2003 and 2004) there were 9000 cases in 2003 and 9900 cases in 2004. There were three nation-wide epidemics which occurred in 1979, 1982 and 1986. However, there were no epidemics between 1987 and 2004.

Until 1980, the prevalence of KD among children in the general population less than 4 years of age was less than 50 per 100,000. Since then, this figure has steadily increased: between 1981 and 1993 the annual incidence rose to 70-90 per 100,000; between 1994 and 1999 it increased to more than 100; and between 1994 and 2002 the number jumped from 119.6 to 151.2 per 100,000.

The average male-female ratio is 1.4:1. The age distribution shows a peak at between 9 and 11 months of age; 50% of affected children are less than 2 years of age and 80% are less than 4. Sibling incidence ranged from 0.7% to 11.4%. The fatality rate was more than 1% until 1974, but fell to 0.1-0.2% between 1974 and 1993. Between 1994 and 2002, the rate was 0.02-0.09% with the exception of 1997 when it was 0.14%.

In 1974, the clinical symptoms and epidemiologic survey results were published in the English language in the journal Pediatrics and this article immediately drew international attention. Subsequently there have been increased reports on KD from many countries, and especially from the United States of America. Research in the USA has shown that the incidence of KD is highest among children of Asian extraction and lowest among children of Caucasian extraction. Reports of KD are more numerous from the more industrialized countries than from the developing countries.

Japanese pediatric cardiologists carried out angiographies on some patients and found coronary artery-aneurysms even in cases that clinically did not indicate such changes. The findings of the autopsied cases and the clinical course matched. "Mucocutaneous lymph node syndrome" was the name established for this symptom complex. This complex was recognized as a vasculitic syndrome and the name gradually changed to Kawasaki Syndrome or Kawasaki Disease.

The above is a short history of the beginnings of Kawasaki Disease.
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