Kawasaki Disease

Epidemiologic Case Definition (Classic Clinical Criteria)

• A) Fever persisting at least 5 days
• B) Presence of at least 4 principal features: (Patients with fever at least 5 days and <4 principal criteria can be diagnosed with Kawasaki disease when coronary artery abnormalities are detected by 2-dimensional echocardiography or angiography) 1) Changes in extremities • Acute: erythema of palms, soles; edema of hands, feet • Subacute: periungual peeling of fingers, toes in wk 2 and 3 2) Polymorphous exanthem 3) Bilateral bulbar conjunctival injection without exudate 4) Erythema and cracking of lips, strawberry tongue, and/or erythema of oral and pharyngeal mucosa 5) Cervical lymphadenopathy (>1.5 cm diameter), usually unilateral
• C) Exclusion of other diseases with similar findings
• *These features do not have to occur concurrently.

Other Clinical and Laboratory Findings

• A) Cardiovascular System • Myocarditis, pericarditis, valvular regurgitation, shock • Coronary artery abnormalities • Aneurysms of medium-sized noncoronary arteries • Peripheral gangrene • Aortic root enlargement
• B) Respiratory System • Peribronchial and interstitial infiltrates on chest radiograph • Pulmonary nodules
• C) Musculoskeletal System • Arthritis, arthralgias (pleocytosis of synovial fluid)
• D) Gastrointestinal Tract • Diarrhea, vomiting, abdominal pain • Hepatitis, jaundice • Hydrops of gallbladder • Pancreatitis
• E) Central Nervous System • Extreme irritability • Aseptic meningitis (pleocytosis of cerebrospinal fluid) • Facial nerve palsy • Sensorineural hearing loss
• F) Genitourinary System • Urethritis/meatitis, hydrocele
• G) Other Findings • Desquamating rash in groin • Retropharyngeal phlegmon • Anterior uveitis by slit-lamp examination • Erythema, induration at bacille Calmette-Guérin inoculation site

Laboratory Findings in Acute Kawasaki Disease

• • Leukocytosis with neutrophilia and immature forms • Elevated erythrocyte sedimentation rate • Elevated C-reactive protein • Anemia • Abnormal plasma lipids • Hypoalbuminemia • Hyponatremia • Thrombocytosis after wk 1 • Sterile pyuria • Elevated serum transaminases • Elevated serum γ-glutamyl transpeptidase • Pleocytosis of cerebrospinal fluid • Leukocytosis in synovial fluid