Why in News?
Kawasaki Disease (KD) is recently in the news due to the publication of a major multicenter study in the New England Journal of Medicine (NEJM). The study, titled "Adjunctive Prednisolone for Kawasaki Disease," has provided critical evidence for clinicians regarding the use of steroids in primary treatment.
About
- Definition: An acute systemic vasculitis (inflammation of blood vessels) that primarily affects medium-sized arteries, most notably the coronary arteries.
- Target Group: Affects children, with 80-90% of cases occurring in those under 5 years of age (peak age 6 months to 2 years).
- Impact: It is the leading cause of acquired heart disease in children in developed nations.
Key Symptoms (The "CREAM" Mnemonic)
A diagnosis typically requires a fever lasting at least 5 days plus 4 of the following 5 criteria:
- C - Conjunctivitis: Redness in both eyes without thick discharge.
- R - Rash: A red, nonspecific rash, often on the trunk or genital area.
- E - Extremity changes: Redness or swelling of palms/soles, followed by skin peeling (desquamation).
- A - Adenopathy: Swollen lymph nodes, usually on one side of the neck (>1.5 cm).
- M - Mucosal changes: Red, dry, or cracked lips and a "strawberry tongue".
Serious Complications
- Coronary Artery Aneurysms (CAA): Ballooning of the heart's arteries, occurring in 20–25% of untreated cases.
- Heart Attack (Myocardial Infarction): Caused by blood clots (thrombosis) forming within an aneurysm.
- Myocarditis: Inflammation of the heart muscle, seen in up to 70% of patients during the acute phase.
Standard Treatment
- IVIG (Intravenous Immunoglobulin): High-dose antibodies given via IV to reduce inflammation and lower CAA risk to less than 5%.
- Aspirin: Used initially in high doses for inflammation and later in low doses to prevent blood clots.
- Biologics: For patients who do not respond to IVIG, newer therapies like Infliximab or Anakinra may be used.
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