Kawasaki Disease Diagnostic Criteria

Children with several days of unexplained fever associated with any of the principal clinical features of Kawasaki Disease (KD).

• The Kawasaki Disease Criteria are widely used for the diagnosis of Kawasaki Disease, also known as mucocutaneous lymph node syndrome, adopted by the American Heart Association (AHA) and endorsed by the American Academy of Pediatrics (APA). (Newburger 2004)
• There is no diagnostic laboratory test. The diagnosis is clinical.

• Clinical findings do not commonly manifest simultaneously and there is no typical order of appearance. Repeated examinations and close history-taking are important.
• Can be confused with other infectious exanthema of childhood, and concurrent viral infections are common. Non-specific symptoms commonly occur in children, including arthritis, vomiting, diarrhea, abdominal pain, irritability, cough, rhinorrhea and decreased oral intake. (Baker 2009)
• Although there is no diagnostic laboratory test, elevated WBC and platelet counts, transaminases, and acute phase reactants, as well as anemia and pyuria, can be suggestive of KD.

Points to keep in mind:

• It is suspected that at least 10% of patients who develop coronary artery (CA) abnormalities fail to meet the criteria for KD. (Sundel 2002). The criteria may fail to diagnose a substantial number of patients.
• Patients suspected of having KD who do not fulfill the diagnostic criteria may have incomplete (atypical) KD. Children with incomplete KD, whose diagnosis is delayed, are more likely to develop CA abnormalities. The AHA and AAP have developed criteria to help diagnose and guide treatment in incomplete KD. (Newburger 2004)

The most common and dangerous long-term sequelae of KD are CA abnormalities (aneurysms or ectasia) that develop in up to 25% of untreated children and may lead to sudden death or ischemic heart disease. (Newburger 2004)