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Vasculitis of unknown etiology.
Cause of Kawasaki disease is unknown, but it may stem from an infection triggering an autoimmune and/or autoinflammatory response in children who are genetically predisposed.
A febrile vasculitis of childhood characterized by fever, rash, conjunctivitis, lymphadenopathy, mucocutaneous lesions and most significantly coronary artery aneurysms.
Kawasaki disease is an acute vasculitis, a systemic disease that mostly involves coronary arteries and typically occurs in children younger than 5 years of age.
Kawasaki disease, or mucocutaneous lymph node syndrome, is a vasculitis that primarily affects medium and large vessels.
Generally affects children younger than 5 years and has replaced rheumatic fever as the primary cause of childhood heart disease in the United States.
Etiology unknown, but suspected to be due to a combination of infectious and autoimmune reasons.
The diagnosis of classic or typical Kawasaki disease is based on clinical criteria established by the American Heart Association.
These criteria include fever for at least 5 days, without plausible alternative explanations: oral mucosal changes: strawberry tongue, erythematous, fissured cracked lips, diffuse erythema of the oral pharynx, bilateral bulbar conjunctival injection without exudate; polymorphous rash with diffuse maculopapular, urticarial, erythroderma, erythema multiforme-like, not vesicular or bullous lesions, changes in the extremities with erythema and indurated edema of the hands and feet, sharp demarcation at the ankles and wrists, periungual desquamation, and cervical lymphadenopathy, >1.5 cm in diameter.
In the presence of 4 or more major features, mainly when redness and swelling of the hands and feet are present, the diagnosis can be made with only 4 days of fever.
Patients who have a fever for 5 or more days and only 3 major clinical features can also receive a classic Kawasaki disease diagnosis when coronary artery disease is detected by 2-dimensional echocardiography or coronary angiography.
No specific diagnostic test exists for Kawasaki disease, and its recognition is based on various combinations of clinical and laboratory findings including: persistent fever, widespread rashes, enlarged lymph nodes, conjunctivitis, changes to the mucous membranes, and swollen hands and feet.
Cardiovascular complications in up to 20% of children with KS.
Considered the most common cause of acquired cardiac disease in young children.
Mortality due to occlusion of coronary artery aneurysms.
Leading cause of acquired heart disease in children younger than 5 years.
Kawasaki’s disease affects children of all ethnic types and susceptibility influenced by genetics.
Highest incidence in Japanese children affecting 175 per 100,000 children younger than 5 years of age with a prevalence of approximately 1 in every 185 Japanese children.
Kawasaki disease is common in East Asia and in children of East Asian descent.
In Japan, there is an incidence of nearly 20-fold higher than in white populations.
U.S. attack rate 20-25 per 100,000 children younger than 5 years, with about 5000 new cases per year.
No diagnostic test for this acute vasculitis.
Usual presentation is abrupt onset of fever, with conjunctival injection without exudates, erythema of the lips, tongue, oral mucosa, rash, swelling and redness of the hands and feet and less often associated with cervical lymph node enlargement.
Laboratory evaluation reveals leucocytosis with increased neutrophils, anemia, elevated erythrocyte sedimentation rate, elevated C-reactive protein and thrombocytosis.
Coronary aneurysms develop in as many as 25% of untreated children and over time leads to ischemia, myocardial infarction and rarely death.
The primary long-term complication is the development of stenosis at the site of an earlier coronary artery aneurysm, which may lead to ischemic heart disease.
Rarely may need to visceral arterial aneurysms and ischemic disease in adulthood.
Thrombocytopenia a risk factor for acute myocardial infarction.
Treatment aspirin and intravenous gamma globulin.