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Kawasaki Disease

  • Kawasaki disease (KD) is the most common vasculitis and cause of acquired heart disease in children.

  • Consider KD in any child under the age of 5 years presenting with prolonged high fevers and conjunctivitis.

  • Intravenous immunoglobulin (IVIG) within 10 days of disease onset is the treatment of choice for KD.

Diagnostic criteria for KD:

  • Bilateral conjunctivitis, nonexudative, often dramatic (85% to 92%).

  • Oropharyngeal manifestations: vertical cracking and fissuring of lips, red pharynx without exudate; “strawberry” tongue (90%).

  • Cervical lymphadenopathy (33% to 66%)—one or more enlarged (>1.5 cm) nodes, usually unilateral and painful.

  • Polymorphic rash involving trunk and extremities (80% to 90%) accentuated in groin (50%)—may be pruritic, never vesicular or bullous.

  • Erythema (painful) of palms and soles, progressing to edema dorsum of hands and feet, and finally periungual desquamation usually 2 to 3 weeks after fever onset (65% to 80%).

  • Fever plus echocardiographic/angiographicdemonstration of coronary artery aneurysms or stenoses.

Treatment for KD:

Conclusive evidence from numerous multicenter, double-blind, placebo-controlled series shows that intravenous immuno globulin (IVIG) at a dose of 2 g/kg given over 12 hours within the first 10 days of illness is the treatment of choice in KD. This is given in conjunction with high-dose aspirin (30 to 50 or 80 to 100 mg/kg/day).

Patients with persistent aneurysms are at risk for accelerated atherosclerosis and/or stenoses requiring stents or bypass surgery.