What is it?
Kawasaki disease, or Kawasaki syndrome, is an acute systemic inflammatory disease that occurs primarily in children under 5 years of age and can lead to serious heart damage and death. It is the leading cause of acquired heart disease in children in industrialized countries in Asia, Europe and North America. Cases are observed all over the world, but it is in Asia that it is the most frequent. It affects about 1 child under 5 years of age per 1,000 in Japan, the country where it was first described in 1967 by pediatrician Tomisaku Kawasaki.
Symptoms of Kawasaki disease
Kawasaki disease is identified when the patient has a high fever of 40 ° C or higher for 5 consecutive days or more, associated with at least 4 of the following 5 clinical signs:
- skin rash ;
- abnormal swelling of the neck glands ( cervical lymphadenopathy );
- irritation and redness of the whites of the eyes (bilateral conjunctivitis);
- irritation / inflammation of the mouth, tongue, lips and throat related to the alteration of the oral mucosa;
- Swelling of the hands and feet.
When some of these signs are not observed, it is considered to be an atypical or incomplete Kawasaki disease, which represents the majority of cases.
Cardiovascular disorders involving the pericardium, myocardium, endocardium, and coronary arteries can manifest as early as the onset of the disease by cardiac arrhythmias such as tachycardia.
The origins of the disease
Kawasaki syndrome was identified late and the doctors originally thought they had a benign syndrome, before realizing that it is actually a systemic inflammation of the vessels of the coronary artery wall, called systemic vasculitis. We still do not know what causes Kawasaki disease. Its clinical and epidemiological characteristics guide researchers towards an infectious cause, but they still fail to identify it. The strongest hypothesis is that Kawasaki disease is caused by an infectious agent producing clinical effects in genetically predisposed individuals, particularly Asians. Another more controversial hypothesis is that Kawasaki’s disease is linked to a so-called “superantigenic” toxin.
Risk factors for Kawasaki disease
Kawasaki disease mainly affects children aged between 6 months and 5 years (85% of patients are under the age of five), and more often boys than girls. The prevalence of the disease is much higher in Asian populations.
It can be complicated by coronary artery aneurysms in 15-30% of untreated cases with different sources that pose a risk for coronary artery disease, myocardial infarction, and sudden death.
Prevention and treatment of Kawasaki disease
The standard treatment is to administer intravenous immunoglobulin and aspirin to prevent inflammation of the coronary artery wall. When it is sufficiently early, it significantly reduces the risk of cardiac sequelae and therefore the appearance of coronary aneurisms and infarction.