Kawasaki Syndrome: Symptoms, Causes & Treatment
Kawasaki syndrome is an acute inflammatory disease of the blood vessels affecting mainly children under 5 years of age, which can damage the heart if left untreated.
Things worth knowing about "Kawasaki syndrome"
Kawasaki syndrome is an acute inflammatory disease of the blood vessels affecting mainly children under 5 years of age, which can damage the heart if left untreated.
What is Kawasaki Syndrome?
Kawasaki syndrome (also known as Kawasaki disease) is an acute febrile vasculitis – an inflammatory disease of medium-sized blood vessels – that primarily affects infants and children under the age of 5. It was first described in 1967 by Japanese pediatrician Tomisaku Kawasaki. Kawasaki disease is the leading cause of acquired heart disease in children in developed countries.
Causes
The exact cause of Kawasaki syndrome remains unknown. Current evidence suggests that an abnormal immune response to an infectious trigger (such as a virus or bacterium) in genetically predisposed children leads to widespread blood vessel inflammation. Key factors under investigation include:
- Genetic predisposition: Higher incidence in children of Asian descent, particularly Japanese and Korean background
- Infectious triggers: Various viruses and bacteria have been proposed as possible triggers
- Immune dysregulation: Abnormal activation of the immune system causes inflammation of the vessel walls
Symptoms
Kawasaki syndrome typically progresses through three phases. The classic diagnostic features include:
- Persistent high fever lasting more than 5 days (usually above 39 °C / 102.2 °F), unresponsive to standard antipyretics
- Bilateral conjunctivitis (red eyes) without discharge
- Changes to the lips and mouth: cracked, red lips, strawberry tongue, redness of the oral mucosa
- Skin rash on the trunk (polymorphous exanthem)
- Changes to hands and feet: redness and swelling, followed by peeling of the skin on fingers and toes
- Cervical lymphadenopathy: swollen lymph nodes in the neck, usually on one side
Additional symptoms may include irritability, abdominal pain, diarrhea, vomiting, and joint pain.
Complications
The most serious complication of Kawasaki syndrome is the development of coronary artery aneurysms – abnormal bulging of the coronary arteries. If left untreated, these can lead to myocardial infarction or sudden cardiac death. With timely treatment, this complication occurs in only about 4% of affected children.
Diagnosis
There is no single definitive test for Kawasaki syndrome. Diagnosis is clinical, based on the characteristic symptoms. Supporting investigations include:
- Blood tests: Elevated inflammatory markers (CRP, ESR), elevated white blood cell count, anemia, elevated platelet count in the later phase
- Echocardiography: Ultrasound of the heart to evaluate the coronary arteries
- ECG: To rule out cardiac complications
- Urinalysis: May show white blood cells in the urine
Treatment
Early treatment is essential to prevent cardiac damage. The standard therapy consists of:
- Intravenous immunoglobulin (IVIG): A single high-dose infusion significantly reduces inflammation and lowers the risk of coronary aneurysms
- Acetylsalicylic acid (Aspirin): High dose for anti-inflammatory effect initially, then low dose to prevent blood clotting
- Corticosteroids: Used in therapy-resistant cases or severe disease
- Biologics: For IVIG-resistant cases, e.g., infliximab or anakinra
Children who develop coronary aneurysms require long-term cardiology follow-up and potentially permanent anticoagulation therapy.
Prognosis
With timely diagnosis and treatment, the prognosis of Kawasaki syndrome is excellent in most cases. The majority of children recover fully without lasting cardiac damage. However, children with coronary aneurysms require ongoing follow-up with a pediatric cardiologist.
References
- McCrindle BW et al. - Diagnosis, Treatment, and Long-Term Management of Kawasaki Disease: A Scientific Statement for Health Professionals From the American Heart Association. Circulation, 2017.
- World Health Organization (WHO) - Kawasaki Disease Information. Available at: https://www.who.int
- Newburger JW et al. - Kawasaki Disease. Journal of the American College of Cardiology, 2016.
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