Kawasaki Disease or Incomplete Kawasaki Disease Clinical Pathway — Emergency Department and Inpatient

History and Physical, Clinical Criteria

Kawasaki disease is an acute self-limited febrile illness of unknown cause primarily affecting children < 5 years old. It is characterized by systemic inflammation in medium-sized arteries and multiple organs and tissues during the acute febrile phase. Major morbidities of KD are related to cardiac sequelae; and it is the leading cause of acquired heart disease in children. In untreated patients, the incidence of coronary artery aneurysms is 25% and is decreased to 4% with the use of timely IVIG. The peak mortality occurs 15-45 days after onset of fever, during which time the coronary artery vasculitis develops with marked elevation of platelets in the presence of a hypercoagulable state. Sudden death may occur years later in children and adults with coronary artery aneurysms and stenoses.

The diagnosis of KD is confirmed when coronary artery aneurysms are identified. These are usually not detected until after the first week of illness; thus a normal echocardiogram in the first week of illness does not rule out the diagnosis of KD. Classic KD is diagnosed in the presence of fever for at least 5 days and at least 4 of the principal clinical features listed below. If > 4 principal clinical features are present, the diagnosis of KD can be made with 4 days of fever. In rare cases, the diagnosis of KD can be made with 3 days of fever when classic clinical features are present.

Although the presence of fever for ≥ 4 days with 4 or 5 principal clinical features establishes the diagnosis of complete KD, these criteria do not identify all children with the illness. The diagnosis of incomplete KD is considered in the presence of: ≥ 4 days fever, ≥ 2 principal clinical features, and supplemental lab findings OR Infants ≤ 6 months with ≥ 7 days of unexplained fever. See: Incomplete Kawasaki Disease

Patient should be assessed for clinical features consistent and inconsistent with KD.

KD should be considered in the differential diagnosis for patients with unexplained prolonged fever and any of the principal clinical features. Principal features of KD may not all be present at the same time, so it is important to assess and obtain history regarding any of the other principal KD features that may have been present but resolved at the time of presentation.

History and Physical
  • Days of fever
  • Symptoms: clinical features, described below
  • Symptoms may not be present at time of presentation, gather history of symptoms
Clinical Features of KD
Principal Clinical Features
See AHA Guidelines Page e935, Figure 2. Clinical features of classic Kawasaki disease
Oral changes Erythema and cracking of lips, strawberry tongue, and/or erythema of oral and pharyngeal mucosa
Conjunctivitis Bilateral bulbar conjunctival injection without exudate
Rash Maculopapular, diffuse erythroderma, or erythema multiforme-like
Extremity changes Erythema and edema of the hands and feet in acute phase and/or periungual desquamation in subacute phase
Lymphadenopathy Cervical lymphadenopathy (≥ 1.5 cm diameter), usually unilateral
Other Clinical Findings
  • Myocarditis, pericarditis, valvular regurgitation, shock
  • Coronary artery abnormalities
  • Aneurysms of medium-sized non-coronary arteries
  • Peripheral gangrene
  • Aortic root enlargement
  • Peribronchial and interstitial infiltrates on chest X-ray
  • Pulmonary nodules
  • Arthritis, arthralgia (pleocytosis of synovial fluid)
  • Diarrhea, vomiting, abdominal pain
  • Hepatitis, jaundice
  • Gallbladder hydrops
  • Pancreatitis
Nervous system
  • Extreme irritability
  • Aseptic meningitis (pleocytosis of cerebrospinal fluid)
  • Facial nerve palsy
  • Sensorineural hearing loss
  • Urethritis/meatitis, hydrocele
  • Desquamating rash in groin
  • Retropharyngeal phlegmon
  • Anterior uveitis by slit lamp examination
  • Erythema and induration at bacillus Calmette-Guerin inoculation site

American Heart Association. 2017 Mar: Circulation 135:00-00. Diagnosis, Treatment, and Long-Term Management of Kawasaki Disease: A Scientific Statement for Health Professionals From the American Heart Association. McCrindle et al.