Kawasaki_Disease_Diagnostic_Criteria_guideline v.1

Kawasaki disease is an acute self-limited vasculitis of childhood that is characterized by fever, bilateral nonexudative conjunctivitis, erythema of the lips and oral mucosa, changes in the extremities, rash, and cervical lymphadenopathy.

Maryam Razavi

maryam.razavi@cambio.se

CambioCDS

To aid clinicians to diagnose Kawasaki Disease.

Use in children with several days of unexplained fever associated with any of the principal clinical features of Kawasaki Disease (KD). Classic clinical features for diagnosis: - Fever persisting at least 5 days -At least 4 of the following 5 principal features: 1. Changes in extremities --Acute: Erythema of palms and soles; edema of hands and feet --Subacute: Periungual peeling of fingers and toes in weeks 2 and 3 2. Polymorphous exanthem 3. Bilateral bulbar conjunctival injection without exudate 4. Changes in lips and oral cavity: Erythema, lips cracking, strawberry tongue, diffuse injection of oral and pharyngeal mucosae 5. Cervical lymphadenopathy (>1.5-cm diameter), usually unilateral - Exclusion of other diseases with similar findings Additionally, the diagnosis is made if any of the following is true: - Fever for ≥5 days and at least 4 of the 5 principal features. - Fever for ≥5 days and <4 principal features when coronary artery disease is detected by 2D echocardiography or coronary angiography. - Day 4 of illness and ≥4 principal features.

Note: Patients suspected of having KD who do not fulfill the diagnostic criteria may have incomplete (atypical) KD.

1. Newburger JW, et al. Diagnosis, Treatment, and Long-Term Management of Kawasaki Disease: A Statement for Health Professionals From the Committee on Rheumatic Fever, Endocarditis and Kawasaki Disease, Council on Cardiovascular Disease in the Young, American Heart Association. Circulation.2004; 110(17), 2747-2771. doi:10.1161/01.cir.0000145143.19711.78 2. Dajani A, et al. Diagnosis and therapy of Kawasaki disease in children. Circulation. 1993; 87, 1776-1780. 3. Baker AL, et al. Associated Symptoms in the Ten Days Before Diagnosis of Kawasaki Disease. The Journal of Pediatrics. 2009; 154(4), 592. doi:10.1016/j.jpeds.2008.10.006 4. Sundel RP. Update on the treatment of kawasaki disease in childhood. Current Rheumatology Reports. 2002; 4(6), 474-482. doi:10.1007/s11926-002-0053-6 5. Kawasaki T. Pediatric acute febrile mucocutaneous lymph node syndrome with characteristic desquamation of fingers and toes: My clinical observation of fifty cases. Pediatr Infect Dis J. 2002; 21, 1-38. 6. Newburger JW, et al. A Single Intravenous Infusion of Gamma Globulin as Compared with Four Infusions in the Treatment of Acute Kawasaki Syndrome. The New England Journal of Medicine. 1991; 24, 1633-1639. 7. Hao S, et al. A Classification Tool for Differentiation of Kawasaki Disease from Other Febrile Illnesses. The Journal of Pediatrics. 2016; 176, 114-120.

OBSERVATION.kawasaki_disease_diagnostic_criteria.v0