From the Guidelines
Kawasaki disease is diagnosed when a patient has fever for at least 5 days plus at least 4 of 5 principal clinical features: polymorphous rash, bilateral conjunctival injection without exudate, changes in the lips and oral cavity, changes in extremities, and cervical lymphadenopathy. 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, as stated in the American Heart Association's scientific statement for health professionals 1.
Diagnostic Criteria
The 5 principal clinical features of Kawasaki disease are:
- Polymorphous rash
- Bilateral conjunctival injection without exudate
- Changes in the lips and oral cavity (reddened or cracked lips, strawberry tongue)
- Changes in extremities (erythema, edema, or desquamation)
- Cervical lymphadenopathy
Treatment
Treatment should begin as soon as the diagnosis is suspected, with the standard regimen consisting of high-dose intravenous immunoglobulin (IVIG) at 2 g/kg given as a single infusion over 10-12 hours, along with high-dose aspirin (80-100 mg/kg/day divided into 4 doses) until the fever resolves. After fever resolution, aspirin is reduced to a low dose (3-5 mg/kg/day) and continued for 6-8 weeks or until platelet counts and inflammatory markers normalize and no coronary abnormalities are present 1. For IVIG-resistant cases (persistent or recurrent fever 36 hours after completing IVIG), a second dose of IVIG is recommended 1. Some patients may require additional therapies such as corticosteroids, infliximab, or cyclosporine.
Monitoring
Cardiac echocardiography should be performed at diagnosis, 1-2 weeks later, and 4-6 weeks after treatment to monitor for coronary abnormalities, as the risk of coronary artery aneurysms is significantly reduced with prompt treatment 1.
From the Research
Diagnostic Criteria for Kawasaki Disease
- The diagnosis of Kawasaki disease is based on clinical criteria, including:
- Fever lasting five or more days
- 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
- Incomplete (atypical) Kawasaki disease occurs in persons with fever lasting five or more days and with two or three of these findings 2, 3
Treatment for Kawasaki Disease
- Treatment for acute disease is:
- Intravenous immunoglobulin (IVIG)
- Aspirin
- If there is no response to treatment, patients are given a second dose of IVIG with or without corticosteroids or other adjunctive treatments 2, 4
- Corticosteroids are effective and well tolerated in Kawasaki disease, both as initial adjunctive treatment in those at high-risk for poor outcome, and as rescue therapy after failed IVIG 5
Diagnostic Imaging
- Transthoracic echocardiography is the diagnostic imaging modality of choice to screen for coronary aneurysms 2
- Computed tomography (CT) or magnetic resonance imaging (MRI) can be utilized to facilitate the diagnosis, especially in cases with head and neck manifestations 6