What are the classic presentation and treatment of Kawasaki disease?

From the Guidelines

Kawasaki disease classically presents as an acute febrile illness in children under 5 years of age with fever lasting at least 5 days, accompanied by five principal features: bilateral non-exudative conjunctivitis, polymorphous rash, cervical lymphadenopathy (usually unilateral and >1.5 cm), changes in the extremities (erythema and edema of hands and feet in acute phase, followed by periungual desquamation in the subacute phase), and changes in the lips and oral cavity (erythema, cracking, strawberry tongue) 1. The diagnosis of classic Kawasaki disease (KD) is based on the presence of ≥5 days of fever and the presence of ≥4 of the 5 principal clinical features, which include:

• Erythema and cracking of lips, strawberry tongue, and/or erythema of oral and pharyngeal mucosa
• Bilateral bulbar conjunctival injection without exudate
• Rash: maculopapular, diffuse erythroderma, or erythema multiforme-like
• Erythema and edema of the hands and feet in acute phase and/or periungual desquamation in subacute phase
• Cervical lymphadenopathy (≥1.5 cm diameter), usually unilateral 1 The standard treatment consists of high-dose intravenous immunoglobulin (IVIG) at 2 g/kg as a single infusion, given within 10 days of fever onset to reduce the risk of coronary artery aneurysms, along with aspirin, initially at high doses (80-100 mg/kg/day divided into four doses) during the acute phase for anti-inflammatory effects, then reduced to low-dose (3-5 mg/kg/day) for antiplatelet effects once the fever resolves 1. This treatment regimen is crucial as Kawasaki disease is the leading cause of acquired heart disease in children in developed countries, with coronary artery aneurysms developing in 25% of untreated cases 1. For IVIG-resistant cases (10-20% of patients), additional therapies may include a second IVIG dose, corticosteroids, infliximab, or cyclosporine 1. Cardiac monitoring with echocardiography is essential at diagnosis and during follow-up to detect coronary abnormalities.

From the Research

Classic Presentation of Kawasaki Disease

The classic presentation of Kawasaki disease is characterized by:

• Fever lasting five or more days
• Accompanied by four out of five findings:
  • Bilateral conjunctival injection
  • Oral changes such as cracked and erythematous lips and strawberry tongue
  • Cervical lymphadenopathy
  • Extremity changes such as erythema or palm and sole desquamation
  • Polymorphous rash 2, 3, 4

Incomplete Kawasaki Disease

Incomplete (atypical) Kawasaki disease occurs in persons with fever lasting five or more days and with two or three of the above findings 2, 4

Treatment of Kawasaki Disease

The standard treatment for acute Kawasaki disease is:

• Intravenous immunoglobulin (IVIG)
• Aspirin 2, 3, 4, 5 If there is no response to treatment, patients may be given a second dose of IVIG with or without corticosteroids or other adjunctive treatments 2, 3, 5

Diagnostic Imaging

Transthoracic echocardiography is the diagnostic imaging modality of choice to screen for coronary aneurysms 2

Long-term Management

The presence and severity of coronary aneurysms and obstruction at diagnosis determine treatment options and the need, periodicity, and intensity of long-term cardiovascular monitoring for potential atherosclerosis 2, 5