From the Guidelines
Kawasaki disease is an acute self-limited vasculitis of childhood characterized by fever, bilateral nonexudative conjunctivitis, erythema of the lips and oral mucosa, changes in the extremities, rash, and cervical lymphadenopathy, with a potential to develop coronary artery aneurysms or ectasia in 15% to 25% of untreated children. The key features of Kawasaki disease include:
- Fever
- Bilateral nonexudative conjunctivitis
- Erythema of the lips and oral mucosa
- Changes in the extremities
- Rash
- Cervical lymphadenopathy The etiology of Kawasaki disease remains unknown, although clinical and epidemiological features strongly suggest an infectious cause 1. The epidemiological features of Kawasaki disease include:
- Age distribution, with 76% of children being 5 years old or younger
- Winter-spring seasonality
- Occurrence of community outbreaks with wave-like geographic spread
- Apparent epidemic cycles
- Higher incidence in boys, with a male-to-female ratio of 1.5 to 1.7:1 The case fatality rate in Kawasaki disease is 0.08% in Japan, with virtually all deaths resulting from cardiac sequelae 1. The diagnosis and management of Kawasaki disease should focus on preventing coronary artery aneurysms and ectasia, as well as reducing the risk of cardiac sequelae and mortality. Further research is needed to understand the exact cause of Kawasaki disease and to develop effective prevention and treatment strategies 1.
From the Research
Definition and Overview of Kawasaki Disease
- Kawasaki disease (KD) is an acute multi-system vasculitis syndrome of unknown etiology occurring mostly in infants and children younger than 5 years of age 2.
- It is the leading cause of acquired heart disease in children in developed countries 2.
- KD is an acute systemic vasculitis which predominantly occurs in childhood but rarely in adulthood 3.
Clinical Features and Diagnosis
- Diagnosis relies on the presence of typical clinical features, but patients may present atypically, increasing the challenge of timely diagnosis for physicians 3.
- Clinical features include persistent fever, rash, and unilateral neck swelling 3.
- Cardiac injury may be evident with elevated troponin T and NT-proBNP, even with normal left ventricular systolic function 3.
Treatment and Management
- Treatment with intravenous immunoglobulins (IVIG) and high-dose aspirin is commonly used 3.
- Despite timely treatment, follow-up computed tomography (CT) coronary angiography may demonstrate aneurysms in the coronary arteries 3.
- The American Heart Association has suggested supplementary criteria for incomplete presentation of KD patients, and various treatment options are available for those with intravenous immunoglobulin treatment failure 2.