Kawasaki Disease (KD): Causes, Symptoms, Diagnosis, and Treatment

Kawasaki disease (KD), also called mucocutaneous lymph node syndrome, is a rare autoimmune condition that causes inflammation in blood vessels, primarily affecting children under age 5. It is the leading cause of acquired heart disease in children in developed countries, often linked to coronary artery complications138.
Causes of Kawasaki Disease
The exact cause remains unknown, but Kawasaki Disease (KD) is thought to involve:
- Immune System Dysregulation: The immune system mistakenly attacks blood vessel walls, triggering inflammation16.
- Genetic Predisposition: Higher risk in children of Asian or Pacific Islander descent15.
- Environmental Triggers: Suspected viral or bacterial infections, though KD is not contagious16.
Symptoms of Kawasaki Disease
Symptoms develop in phases:
- Acute Phase (Days 1–10):
- Subacute Phase (Weeks 2–3):
Atypical KD: Fever with fewer than four classic symptoms, requiring urgent evaluation56.
Diagnosis
Diagnosis is based on clinical criteria and exclusion of other conditions (e.g., scarlet fever, MIS-C):
- Fever ≥5 days + ≥4 classic symptoms25.
- Imaging:
- Echocardiogram: Detects coronary artery aneurysms (most severe complication)38.
- Blood Tests: Elevated CRP, ESR, or white blood cell count67.
Treatment
Treatment aims to reduce inflammation and prevent cardiac damage:
- Intravenous Immunoglobulin (IVIG):
- Aspirin:
- High-dose during acute phase (anti-inflammatory).
- Low-dose afterward (antiplatelet)35.
- Monitoring:
- Echocardiograms: Repeated to check for coronary artery changes8.
Complications
- Coronary Artery Aneurysms: Risk increases with delayed treatment38.
- Myocarditis: Inflammation of the heart muscle36.
- Long-Term Heart Disease: Aneurysms may persist, requiring lifelong monitoring58.
Risk Factors
- Age: Most common in children <5 years16.
- Sex: Slightly more frequent in boys15.
- Ethnicity: Higher incidence in Asian/Pacific Islander populations15.
Prevention
No proven prevention, but early diagnosis and treatment reduce complications68.
Conclusion
Kawasaki disease is a medical emergency requiring prompt intervention to prevent heart damage. While its cause is unclear, timely IVIG and aspirin therapy significantly improve outcomes.
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References
- MedlinePlus: Kawasaki Disease
- Mayo Clinic: Kawasaki Disease
- Wikipedia: Kawasaki Disease
- WebMD: Kawasaki Disease
- Johns Hopkins: Kawasaki Disease
- Cincinnati Children’s: Kawasaki Disease
- Cleveland Clinic: Kawasaki Disease
- CDC: Kawasaki Disease