Kawasaki Syndrome: Causes, Symptoms and Treatment
Kawasaki syndrome is an acute inflammatory disease of the blood vessels in children, primarily affecting those under 5 years old, and can cause serious heart complications if left untreated.
Things worth knowing about "Kawasaki syndrome"
Kawasaki syndrome is an acute inflammatory disease of the blood vessels in children, primarily affecting those under 5 years old, and can cause serious heart complications if left untreated.
What is Kawasaki Syndrome?
Kawasaki syndrome (also known as Kawasaki disease) is an acute febrile illness characterized by systemic inflammation of medium-sized blood vessels, particularly the coronary arteries (the blood vessels supplying the heart). It is one of the most common acquired heart diseases in children in developed countries. The condition was first described in 1967 by Japanese physician Tomisaku Kawasaki. It predominantly affects children under the age of 5, though older children can also be affected.
Causes
The exact cause of Kawasaki syndrome remains unknown. Researchers believe it results from an interaction between genetic predisposition and an excessive immune response triggered by an infectious agent such as a virus or bacterium. The following factors are under investigation:
- Genetic susceptibility (more common in children of Asian descent)
- Preceding respiratory infections
- Dysregulation of the immune system leading to excessive inflammation
- Seasonal clustering (late winter and spring)
Symptoms
Kawasaki syndrome typically progresses in phases. The classical diagnostic criteria include:
- Persistent fever lasting more than 5 days (usually above 38.5 °C / 101.3 °F)
- Conjunctivitis: Bilateral, non-purulent (non-draining) redness of the eyes
- Oral changes: Red, cracked lips, strawberry tongue, and reddened throat
- Skin rash: Polymorphous exanthem (variable skin rash affecting the body)
- Changes in the extremities: Redness and swelling of the hands and feet, followed by peeling (desquamation) of the fingertips and toes
- Cervical lymphadenopathy: Swollen lymph nodes in the neck (often unilateral)
Additional symptoms may include irritability, abdominal pain, diarrhea, joint pain, and in severe cases, heart rhythm disturbances.
Diagnosis
The diagnosis of Kawasaki syndrome is made clinically, as there is no specific diagnostic test. Diagnosis is confirmed when fever lasts more than 5 days and at least 4 of the 5 classical criteria are met. The following additional investigations are performed:
- Blood tests: Elevated inflammatory markers (CRP, ESR, white blood cell count), thrombocytosis (elevated platelet count) in the later phase
- Echocardiography: Ultrasound of the heart to detect coronary artery aneurysms (abnormal bulging of the coronary arteries)
- ECG: To rule out cardiac arrhythmias
- Urinalysis: Sterile pyuria (white blood cells in urine without bacterial infection) is frequently found
An incomplete (atypical) form of Kawasaki syndrome also exists, where not all criteria are met, making diagnosis more challenging.
Treatment
Early treatment is essential to prevent damage to the coronary arteries. The standard treatment includes:
- Intravenous immunoglobulin (IVIG): A single high-dose infusion significantly reduces the risk of coronary artery aneurysms and should ideally be given within the first 10 days of illness.
- Aspirin (acetylsalicylic acid): Used in high doses during the acute phase for its anti-inflammatory and fever-reducing effects, then continued in low doses to prevent blood clots (antiplatelet therapy).
- Corticosteroids: May be used in IVIG-resistant cases or particularly severe presentations.
- Biologics: In treatment-resistant cases, agents such as infliximab or ciclosporin may be considered.
Outlook and Prognosis
With prompt treatment, most children make a full recovery. The greatest risk is the development of coronary artery aneurysms, which occur in up to 25% of untreated patients. With IVIG therapy, this risk is reduced to below 5%. Children with confirmed aneurysms require long-term cardiac follow-up care.
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;135(17):e927-e999.
- Newburger JW et al. - Diagnosis, Treatment, and Long-Term Management of Kawasaki Disease. Pediatrics. 2004;114(6):1708-1733.
- World Health Organization (WHO) - Kawasaki Disease Overview. Available at: www.who.int
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